isodesmosine profile

Isodesmosine is a cross-linking amino acid found exclusively in elastin, a protein that provides elasticity and resilience to tissues such as skin, lungs, and blood vessels. It is formed through a complex enzymatic process involving the condensation of three lysine residues. Isodesmosine plays a crucial role in the formation of elastin fibers, contributing to their unique mechanical properties. Its presence is a hallmark of elastin, making it a valuable marker for the identification and quantification of this protein. Researchers study isodesmosine to understand elastin biosynthesis, degradation, and its role in various diseases affecting connective tissues. For example, changes in isodesmosine levels are associated with aging, skin disorders, and cardiovascular diseases. The synthesis of isodesmosine involves a series of enzymatic steps, starting with the oxidation of lysine residues to form allysine. The allysine residues then undergo a complex series of reactions to form the isodesmosine cross-link. Isodesmosine is a relatively stable molecule, but it can be degraded by enzymes known as elastases. The degradation of isodesmosine contributes to the loss of elastin integrity and can contribute to the development of diseases such as emphysema.'

Isodesmosine: 2-(4-Amino-4-carboxybutyl)-1-(5-amino-5-carboxypentyl)-3,5-bis(3-amino-3-carboxypropyl)pyridinium. A rare amino acid found in elastin, formed by condensation of four molecules of lysine into a pyridinium ring. [Medical Subject Headings (MeSH), National Library of Medicine, extracted Dec-2023]

isodesmosine : A pyridinium ion obtained by formal condensation of four molecules of lysine. [Chemical Entities of Biological Interest (ChEBI), Hastings J, Owen G, Dekker A, Ennis M, Kale N, Muthukrishnan V, Turner S, Swainston N, Mendes P, Steinbeck C. (2016). ChEBI in 2016: Improved services and an expanding collection of metabolites. Nucleic Acids Res]

ID Source	ID
PubMed CID	13811
CHEBI ID	64366
SCHEMBL ID	12707032
MeSH ID	M0011755

Synonym
991-01-5
pyridinium, 2-(4-amino-4-carboxybutyl)-1-(5-amino-5-carboxypentyl)-3,5-bis(3-amino-3-carboxypropyl)-
isodesmosine
2-amino-6-[3,5-bis(3-amino-4-hydroxy-4-oxobutyl)-2-(4-amino-5-hydroxy-5-oxopentyl)pyridin-1-ium-1-yl]hexanoic acid
2-(4-amino-4-carboxybutyl)-1-(5-amino-5-carboxypentyl)-3,5-bis(3-amino-3-carboxypropyl)pyridinium
6-[2-(4-amino-4-carboxybutyl)-3,5-bis(3-amino-3-carboxypropyl)pyridinium-1-yl]norleucine
CHEBI:64366
SCHEMBL12707032
3,5-bis(3-amino-3-carboxypropyl)-2-(4-amino-4-carboxybutyl)-1-(5-amino-5-carboxypentyl)pyridin-1-ium
iso-desmosin
2-(4-amino-4-carboxybutyl)-1-(5-amino-5-carboxypentyl)-3,5-bis(3-amino-3-carboxypropyl)-pyridinium
Q4198750
2-amino-6-[2-(4-amino-4-carboxybutyl)-3,5-bis(3-amino-3-carboxypropyl)pyridin-1-ium-1-yl]hexanoic acid
mfcd00211039
AT34388
isodesmosine (from bovine neck ligament)

Excerpt	Reference	Relevance
" In a 2-week, randomized, double-blind trial, 8 patients receiving 150 kDa HA (mean molecular weight) and 3 others given placebo did not show significant adverse effects with regard to spirometry, electrocardiograms, and hematological indices."	( A pilot clinical trial to determine the safety and efficacy of aerosolized hyaluronan as a treatment for COPD. Cantor, J; Ma, S; Turino, G, 2017)	0.46

Role	Description
biomarker	A substance used as an indicator of a biological state.
[role information is derived from Chemical Entities of Biological Interest (ChEBI), Hastings J, Owen G, Dekker A, Ennis M, Kale N, Muthukrishnan V, Turner S, Swainston N, Mendes P, Steinbeck C. (2016). ChEBI in 2016: Improved services and an expanding collection of metabolites. Nucleic Acids Res]

Class	Description
pyridinium ion
lysine derivative	An amino acid derivative resulting from reaction of lysine at the amino group or the carboxy group, or from the replacement of any hydrogen of lysine by a heteroatom. The definition normally excludes peptides containing lysine residues.
[compound class information is derived from Chemical Entities of Biological Interest (ChEBI), Hastings J, Owen G, Dekker A, Ennis M, Kale N, Muthukrishnan V, Turner S, Swainston N, Mendes P, Steinbeck C. (2016). ChEBI in 2016: Improved services and an expanding collection of metabolites. Nucleic Acids Res]

Timeframe	Studies, This Drug (%)	All Drugs %
pre-1990	54 (36.24)	18.7374
1990's	27 (18.12)	18.2507
2000's	23 (15.44)	29.6817
2010's	42 (28.19)	24.3611
2020's	3 (2.01)	2.80
[information is prepared from research data collected from National Library of Medicine (NLM), extracted Dec-2023]

Publication Type	This drug (%)	All Drugs (%)
Trials	2 (1.31%)	5.53%
Reviews	9 (5.88%)	6.00%
Case Studies	1 (0.65%)	4.05%
Observational	0 (0.00%)	0.25%
Other	141 (92.16%)	84.16%
[information is prepared from research data collected from National Library of Medicine (NLM), extracted Dec-2023]

Substance	Relationship Strength	Studies	Trials	Classes	Roles
citric acid, anhydrous Citric Acid: A key intermediate in metabolism. It is an acid compound found in citrus fruits. The salts of citric acid (citrates) can be used as anticoagulants due to their calcium chelating ability.. citric acid : A tricarboxylic acid that is propane-1,2,3-tricarboxylic acid bearing a hydroxy substituent at position 2. It is an important metabolite in the pathway of all aerobic organisms.	1.96	1	0	tricarboxylic acid	antimicrobial agent; chelator; food acidity regulator; fundamental metabolite
hydrochloric acid Hydrochloric Acid: A strong corrosive acid that is commonly used as a laboratory reagent. It is formed by dissolving hydrogen chloride in water. GASTRIC ACID is the hydrochloric acid component of GASTRIC JUICE.. hydrogen chloride : A mononuclear parent hydride consisting of covalently bonded hydrogen and chlorine atoms.	2.06	1	0	chlorine molecular entity; gas molecular entity; hydrogen halide; mononuclear parent hydride	mouse metabolite
2-aminoadipic acid 2-Aminoadipic Acid: A metabolite in the principal biochemical pathway of lysine. It antagonizes neuroexcitatory activity modulated by the glutamate receptor, N-METHYL-D-ASPARTATE; (NMDA).. 2-aminoadipic acid : An alpha-amino acid that is adipic acid bearing a single amino substituent at position 2. An intermediate in the formation of lysine.	2	1	0	amino dicarboxylic acid; dicarboxylic fatty acid; non-proteinogenic alpha-amino acid	Caenorhabditis elegans metabolite; mammalian metabolite
propionaldehyde propionaldehyde: may cause respiratory irritation; RN given refers to parent cpd; structure. propanal : An aldehyde that consists of ethane bearing a formyl substituent. The parent of the class of propanals.	2	1	0	alpha-CH2-containing aldehyde; propanals	Escherichia coli metabolite
formaldehyde paraform: polymerized formaldehyde; RN given refers to parent cpd; used in root canal therapy	2.48	2	0	aldehyde; one-carbon compound	allergen; carcinogenic agent; disinfectant; EC 3.5.1.4 (amidase) inhibitor; environmental contaminant; Escherichia coli metabolite; mouse metabolite; Saccharomyces cerevisiae metabolite
phenol [no description available]	1.97	1	0	phenols	antiseptic drug; disinfectant; human xenobiotic metabolite; mouse metabolite
pyridine azine : An organonitrogen compound of general structure RCH=N-N=CHR or RR'C=N-N=CRR'.	7.1	1	0	azaarene; mancude organic heteromonocyclic parent; monocyclic heteroarene; pyridines	environmental contaminant; NMR chemical shift reference compound
aminopropionitrile Aminopropionitrile: Reagent used as an intermediate in the manufacture of beta-alanine and pantothenic acid.	2.4	2	0	aminopropionitrile	antineoplastic agent; antirheumatic drug; collagen cross-linking inhibitor; plant metabolite
iproniazid [no description available]	1.95	1	0	carbohydrazide; pyridines
rofecoxib [no description available]	2.08	1	0	butenolide; sulfone	analgesic; cyclooxygenase 2 inhibitor; non-steroidal anti-inflammatory drug
hydroxyproline Hydroxyproline: A hydroxylated form of the imino acid proline. A deficiency in ASCORBIC ACID can result in impaired hydroxyproline formation.. hydroxyproline : A proline derivative that is proline substituted by at least one hydroxy group.	3.24	6	0	4-hydroxyproline; L-alpha-amino acid zwitterion	human metabolite; mouse metabolite; plant metabolite
penicillamine Penicillamine: 3-Mercapto-D-valine. The most characteristic degradation product of the penicillin antibiotics. It is used as an antirheumatic and as a chelating agent in Wilson's disease.. penicillamine : An alpha-amino acid having the structure of valine substituted at the beta position with a sulfanyl group.	2.9	4	0	non-proteinogenic alpha-amino acid; penicillamine	antirheumatic drug; chelator; copper chelator; drug allergen
lysine Lysine: An essential amino acid. It is often added to animal feed.. lysine : A diamino acid that is caproic (hexanoic) acid bearing two amino substituents at positions 2 and 6.. L-lysine : An L-alpha-amino acid; the L-isomer of lysine.	8.91	13	0	aspartate family amino acid; L-alpha-amino acid zwitterion; L-alpha-amino acid; lysine; organic molecular entity; proteinogenic amino acid	algal metabolite; anticonvulsant; Escherichia coli metabolite; human metabolite; micronutrient; mouse metabolite; nutraceutical; plant metabolite; Saccharomyces cerevisiae metabolite
tyrosine Tyrosine: A non-essential amino acid. In animals it is synthesized from PHENYLALANINE. It is also the precursor of EPINEPHRINE; THYROID HORMONES; and melanin.. tyrosine : An alpha-amino acid that is phenylalanine bearing a hydroxy substituent at position 4 on the phenyl ring.	6.97	1	0	amino acid zwitterion; erythrose 4-phosphate/phosphoenolpyruvate family amino acid; L-alpha-amino acid; proteinogenic amino acid; tyrosine	EC 1.3.1.43 (arogenate dehydrogenase) inhibitor; fundamental metabolite; micronutrient; nutraceutical
valine Valine: A branched-chain essential amino acid that has stimulant activity. It promotes muscle growth and tissue repair. It is a precursor in the penicillin biosynthetic pathway.. valine : A branched-chain amino acid that consists of glycine in which one of the hydrogens attached to the alpha-carbon is substituted by an isopropyl group.. L-valine : The L-enantiomer of valine.	1.95	1	0	L-alpha-amino acid zwitterion; L-alpha-amino acid; proteinogenic amino acid; pyruvate family amino acid; valine	algal metabolite; Escherichia coli metabolite; human metabolite; micronutrient; mouse metabolite; nutraceutical; Saccharomyces cerevisiae metabolite
tryptophan Tryptophan: An essential amino acid that is necessary for normal growth in infants and for NITROGEN balance in adults. It is a precursor of INDOLE ALKALOIDS in plants. It is a precursor of SEROTONIN (hence its use as an antidepressant and sleep aid). It can be a precursor to NIACIN, albeit inefficiently, in mammals.. tryptophan : An alpha-amino acid that is alanine bearing an indol-3-yl substituent at position 3.	6.96	1	0	erythrose 4-phosphate/phosphoenolpyruvate family amino acid; L-alpha-amino acid zwitterion; L-alpha-amino acid; proteinogenic amino acid; tryptophan zwitterion; tryptophan	antidepressant; Escherichia coli metabolite; human metabolite; micronutrient; mouse metabolite; nutraceutical; plant metabolite; Saccharomyces cerevisiae metabolite
arginine Arginine: An essential amino acid that is physiologically active in the L-form.. arginine : An alpha-amino acid that is glycine in which the alpha-is substituted by a 3-guanidinopropyl group.	2.01	1	0	arginine; glutamine family amino acid; L-alpha-amino acid; proteinogenic amino acid	biomarker; Escherichia coli metabolite; micronutrient; mouse metabolite; nutraceutical
phenylisothiocyanate phenylisothiocyanate: structure. phenyl isothiocyanate : An isothiocyanate having a phenyl group attached to the nitrogen; used for amino acid sequencing in the Edman degradation.	6.97	1	0	isothiocyanate	allergen; reagent
3-hydroxypyridine 3-hydroxypyridine: RN given refeirs to parent cpd. 3-pyridinol : A monohydroxypyridine that is pyridine in which the hydrogen at position 3 has been replaced by a hydroxy group. It has been detected as a thermal degradation product from the smoke of the burning leaves of Salvia divinorum, a Mexican psychoactive plant.	1.96	1	0	monohydroxypyridine
n-butylamine n-butylamine: RN given refers to parent cpd. butan-1-amine : A primary aliphatic amine that is butane substituted by an amino group at position 1.	2	1	0	primary aliphatic amine
cyanogen bromide Cyanogen Bromide: Cyanogen bromide (CNBr). A compound used in molecular biology to digest some proteins and as a coupling reagent for phosphoroamidate or pyrophosphate internucleotide bonds in DNA duplexes.	1.97	1	0
norleucine Norleucine: An unnatural amino acid that is used experimentally to study protein structure and function. It is structurally similar to METHIONINE, however it does not contain SULFUR.. L-norleucine : A non-proteinogenic L-alpha-amino acid comprising hexanoic acid carrying an amino group at C-2. It does not occur naturally.	1.95	1	0	2-aminohexanoic acid; L-alpha-amino acid zwitterion; non-proteinogenic L-alpha-amino acid
deuterium Deuterium: The stable isotope of hydrogen. It has one neutron and one proton in the nucleus.	3.87	2	1	dihydrogen
ozone Ozone: The unstable triatomic form of oxygen, O3. It is a powerful oxidant that is produced for various chemical and industrial uses. Its production is also catalyzed in the ATMOSPHERE by ULTRAVIOLET RAY irradiation of oxygen or other ozone precursors such as VOLATILE ORGANIC COMPOUNDS and NITROGEN OXIDES. About 90% of the ozone in the atmosphere exists in the stratosphere (STRATOSPHERIC OZONE).. ozone : An elemental molecule with formula O3. An explosive, pale blue gas (b.p. -112degreeC) that has a characteristic, pungent odour, it is continuously produced in the upper atmosphere by the action of solar ultraviolet radiation on atmospheric oxygen. It is an antimicrobial agent used in the production of bottled water, as well as in the treatment of meat, poultry and other foodstuffs.	2.66	3	0	elemental molecule; gas molecular entity; reactive oxygen species; triatomic oxygen	antiseptic drug; disinfectant; electrophilic reagent; greenhouse gas; mutagen; oxidising agent; tracer
propionic anhydride [no description available]	2.08	1	0
nicotine (S)-nicotine : A 3-(1-methylpyrrolidin-2-yl)pyridine in which the chiral centre has S-configuration. The naturally occurring and most active enantiomer of nicotine, isolated from Nicotiana tabacum.	2.42	2	0	3-(1-methylpyrrolidin-2-yl)pyridine	anxiolytic drug; biomarker; immunomodulator; mitogen; neurotoxin; nicotinic acetylcholine receptor agonist; peripheral nervous system drug; phytogenic insecticide; plant metabolite; psychotropic drug; teratogenic agent; xenobiotic
fibrinogen Fibrinogen: Plasma glycoprotein clotted by thrombin, composed of a dimer of three non-identical pairs of polypeptide chains (alpha, beta, gamma) held together by disulfide bonds. Fibrinogen clotting is a sol-gel change involving complex molecular arrangements: whereas fibrinogen is cleaved by thrombin to form polypeptides A and B, the proteolytic action of other enzymes yields different fibrinogen degradation products.. D-iditol : The D-enantiomer of iditol.	4.12	2	0	iditol	fungal metabolite
histidinoalanine histidinoalanine: cross-linking amino acid in calcified tissue collagen; RN given refers to (L)-isomer	2.36	2	0	dipeptide zwitterion; dipeptide	metabolite
pyridinoline pyridinoline: 3-hydroxypyridinium derivative collagen crosslink; structure	5.11	14	0	organonitrogen compound; organooxygen compound
deoxypyridinoline deoxypyridinoline: structure given in first source	2.9	4	0
pentosidine pentosidine: structure given in first source. pentosidine : An imidazopyridine having norleucine and ornithine residues attached via their side-chains at the 4- and 2-positions respectively.	2.01	1	0	imidazopyridine; non-proteinogenic L-alpha-amino acid	biomarker; cross-linking reagent
proline Proline: A non-essential amino acid that is synthesized from GLUTAMIC ACID. It is an essential component of COLLAGEN and is important for proper functioning of joints and tendons.. proline : An alpha-amino acid that is pyrrolidine bearing a carboxy substituent at position 2.	1.95	1	0	amino acid zwitterion; glutamine family amino acid; L-alpha-amino acid; proline; proteinogenic amino acid	algal metabolite; compatible osmolytes; Escherichia coli metabolite; micronutrient; mouse metabolite; nutraceutical; Saccharomyces cerevisiae metabolite
allysine L-allysine : An optically active form of allysine having L-configuration.	2	1	0	allysine; amino acid zwitterion; aminoadipate semialdehyde; non-proteinogenic L-alpha-amino acid	human metabolite; mouse metabolite; Saccharomyces cerevisiae metabolite
lysinenorleucine lysinenorleucine: RN given refers to (S)-isomer	1.96	1	0
elastin [no description available]	11.42	103	1	oligopeptide
cotinine Cotinine: The N-glucuronide conjugate of cotinine is a major urinary metabolite of NICOTINE. It thus serves as a biomarker of exposure to tobacco SMOKING. It has CNS stimulating properties.. (-)-cotinine : An N-alkylpyrrolidine that consists of N-methylpyrrolidinone bearing a pyridin-3-yl substituent at position C-5 (the 5S-enantiomer). It is an alkaloid commonly found in Nicotiana tabacum.	2.06	1	0	N-alkylpyrrolidine; pyridines; pyrrolidin-2-ones; pyrrolidine alkaloid	antidepressant; biomarker; human xenobiotic metabolite; plant metabolite
lithium Lithium: An element in the alkali metals family. It has the atomic symbol Li, atomic number 3, and atomic weight [6.938; 6.997]. Salts of lithium are used in treating BIPOLAR DISORDER.	1.96	1	0	alkali metal atom
hydroxylysine Hydroxylysine: A hydroxylated derivative of the amino acid LYSINE that is present in certain collagens.. hydroxylysine : A hydroxy-amino acid in which the amino acid specified is lysine. A "closed" class.. erythro-5-hydroxy-L-lysine : A 5-hydroxylysine consisting of L-lysine having an (R)-hydroxy group at the 5-position.. 5-hydroxylysine : A hydroxylysine that is lysine substituted by a hydroxy group at position 5.	7.68	3	0	5-hydroxylysine; hydroxy-L-lysine	human metabolite
sodium dodecyl sulfate Sodium Dodecyl Sulfate: An anionic surfactant, usually a mixture of sodium alkyl sulfates, mainly the lauryl; lowers surface tension of aqueous solutions; used as fat emulsifier, wetting agent, detergent in cosmetics, pharmaceuticals and toothpastes; also as research tool in protein biochemistry.. sodium dodecyl sulfate : An organic sodium salt that is the sodium salt of dodecyl hydrogen sulfate.	1.96	1	0	organic sodium salt	detergent; protein denaturant
alpha-chymotrypsin Chymotrypsin: A serine endopeptidase secreted by the pancreas as its zymogen, CHYMOTRYPSINOGEN and carried in the pancreatic juice to the duodenum where it is activated by TRYPSIN. It selectively cleaves aromatic amino acids on the carboxyl side.	1.96	1	0
osteoprotegerin Osteoprotegerin: A secreted member of the TNF receptor superfamily that negatively regulates osteoclastogenesis. It is a soluble decoy receptor of RANK LIGAND that inhibits both CELL DIFFERENTIATION and function of OSTEOCLASTS by inhibiting the interaction between RANK LIGAND and RECEPTOR ACTIVATOR OF NUCLEAR FACTOR-KAPPA B.	3.18	1	0	long-chain fatty acid
vitamin k semiquinone radical vitamin K semiquinone radical: found in active preparations of vitamin K-dependent carboxylase. vitamin K : Any member of a group of fat-soluble 2-methyl-1,4-napthoquinones that exhibit biological activity against vitamin K deficiency. Vitamin K is required for the synthesis of prothrombin and certain other blood coagulation factors.	2.15	1	0
tiotropium bromide Tiotropium Bromide: A scopolamine derivative and CHOLINERGIC ANTAGONIST that functions as a BRONCHODILATOR AGENT. It is used in the treatment of CHRONIC OBSTRUCTIVE PULMONARY DISEASE.. tiotropium bromide : An organic bromide salt having (1alpha,2beta,4beta,5alpha,7beta)-7-[(hydroxydi-2-thienylacetyl)oxy]-9,9-dimethyl-3-oxa-9-azoniatricyclo[3.3.1.0(2,4)]nonane as the counterion. Used (in the form of the hydrate) for maintenance treatment of airflow obstruction in patients with chronic obstructive pulmonary disease.	2.05	1	0
beta-aminopropionitrile fumarate beta-aminopropionitrile fumarate: RN given refers to unspecified fumarate	1.96	1	0
jaw [no description available]	2.03	1	0	indolecarboxamide
desmosine Desmosine: A rare amino acid found in elastin, formed by condensation of four molecules of lysine into a pyridinium ring.	12.52	140	2	aromatic amino acid
piperidines Piperidines: A family of hexahydropyridines.	1.99	1	0
natriuretic peptide, brain Natriuretic Peptide, Brain: A PEPTIDE that is secreted by the BRAIN and the HEART ATRIA, stored mainly in cardiac ventricular MYOCARDIUM. It can cause NATRIURESIS; DIURESIS; VASODILATION; and inhibits secretion of RENIN and ALDOSTERONE. It improves heart function. It contains 32 AMINO ACIDS.	3.18	1	0	polypeptide

Condition	Relationship Strength	Studies	Trials
Centriacinar Emphysema [description not available]	5.74	20	0
Skin Aging The process of aging due to changes in the structure and elasticity of the skin over time. It may be a part of physiological aging or it may be due to the effects of ultraviolet radiation, usually through exposure to sunlight.	2.6	1	0
Airflow Obstruction, Chronic [description not available]	10.23	28	2
Pulmonary Disease, Chronic Obstructive A disease of chronic diffuse irreversible airflow obstruction. Subcategories of COPD include CHRONIC BRONCHITIS and PULMONARY EMPHYSEMA.	10.23	28	2
Disease, Pulmonary [description not available]	2.4	2	0
Deficiency, Vitamin K [description not available]	2.15	1	0
Lung Diseases Pathological processes involving any part of the LUNG.	2.4	2	0
Vitamin K Deficiency A nutritional condition produced by a deficiency of VITAMIN K in the diet, characterized by an increased tendency to hemorrhage (HEMORRHAGIC DISORDERS). Such bleeding episodes may be particularly severe in newborn infants. (From Cecil Textbook of Medicine, 19th ed, p1182)	2.15	1	0
Disease Models, Animal Naturally-occurring or experimentally-induced animal diseases with pathological processes analogous to human diseases.	3.13	5	0
alpha 1-Antitrypsin Deficiency Deficiency of the protease inhibitor ALPHA 1-ANTITRYPSIN that manifests primarily as PULMONARY EMPHYSEMA and LIVER CIRRHOSIS.	4.46	8	0
Cardiovascular Diseases Pathological conditions involving the CARDIOVASCULAR SYSTEM including the HEART; the BLOOD VESSELS; or the PERICARDIUM.	3.91	4	0
Infections, Pseudomonas [description not available]	2.11	1	0
Pseudomonas Infections Infections with bacteria of the genus PSEUDOMONAS.	2.11	1	0
Alveolitis, Fibrosing [description not available]	2.41	2	0
Emphysema A pathological accumulation of air in tissues or organs.	2.91	4	0
Pulmonary Fibrosis A process in which normal lung tissues are progressively replaced by FIBROBLASTS and COLLAGEN causing an irreversible loss of the ability to transfer oxygen into the bloodstream via PULMONARY ALVEOLI. Patients show progressive DYSPNEA finally resulting in death.	7.41	2	0
Smoking Cessation Discontinuing the habit of SMOKING.	2.05	1	0
Cystic Fibrosis of Pancreas [description not available]	2.92	4	0
Innate Inflammatory Response [description not available]	3.39	2	0
Cystic Fibrosis An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION.	2.92	4	0
Inflammation A pathological process characterized by injury or destruction of tissues caused by a variety of cytologic and chemical reactions. It is usually manifested by typical signs of pain, heat, redness, swelling, and loss of function.	3.39	2	0
Lymphangiomyomatosis [description not available]	2.05	1	0
Cancer of Lung [description not available]	3.39	2	0
Lung Neoplasms Tumors or cancer of the LUNG.	3.39	2	0
Sensitivity and Specificity Binary classification measures to assess test results. Sensitivity or recall rate is the proportion of true positives. Specificity is the probability of correctly determining the absence of a condition. (From Last, Dictionary of Epidemiology, 2d ed)	3.15	5	0
Lymphangioleiomyomatosis A disease characterized by the progressive invasion of SMOOTH MUSCLE CELLS into the LYMPHATIC VESSELS, and the BLOOD VESSELS. The majority of the cases occur in the LUNGS of women of child-bearing age, eventually blocking the flow of air, blood, and lymph. The common symptom is shortness of breath (DYSPNEA).	2.05	1	0
Hyperhomocysteinemia Condition in which the plasma levels of homocysteine and related metabolites are elevated (	2.06	1	0
Bare Lymphocyte Syndrome [description not available]	2.06	1	0
Severe Combined Immunodeficiency Group of rare congenital disorders characterized by impairment of both humoral and cell-mediated immunity, leukopenia, and low or absent antibody levels. It is inherited as an X-linked or autosomal recessive defect. Mutations occurring in many different genes cause human Severe Combined Immunodeficiency (SCID).	2.06	1	0
Disease Exacerbation [description not available]	2.99	1	0
Airway Remodeling The structural changes in the number, mass, size and/or composition of the airway tissues.	3	1	0
Aging The gradual irreversible changes in structure and function of an organism that occur as a result of the passage of time.	4.28	7	0
Cachexia General ill health, malnutrition, and weight loss, usually associated with chronic disease.	3	1	0
Body Weight The mass or quantity of heaviness of an individual. It is expressed by units of pounds or kilograms.	3.82	2	1
Delayed Effects, Prenatal Exposure [description not available]	2.01	1	0
Muscle Contraction A process leading to shortening and/or development of tension in muscle tissue. Muscle contraction occurs by a sliding filament mechanism whereby actin filaments slide inward among the myosin filaments.	2.01	1	0
Pregnancy The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH.	2.88	4	0
Apoplexy [description not available]	2.01	1	0
Blood Pressure, High [description not available]	2.41	2	0
Hypertension Persistently high systemic arterial BLOOD PRESSURE. Based on multiple readings (BLOOD PRESSURE DETERMINATION), hypertension is currently defined as when SYSTOLIC PRESSURE is consistently greater than 140 mm Hg or when DIASTOLIC PRESSURE is consistently 90 mm Hg or more.	2.41	2	0
Hypertrophy General increase in bulk of a part or organ due to CELL ENLARGEMENT and accumulation of FLUIDS AND SECRETIONS, not due to tumor formation, nor to an increase in the number of cells (HYPERPLASIA).	2.01	1	0
Stroke A group of pathological conditions characterized by sudden, non-convulsive loss of neurological function due to BRAIN ISCHEMIA or INTRACRANIAL HEMORRHAGES. Stroke is classified by the type of tissue NECROSIS, such as the anatomic location, vasculature involved, etiology, age of the affected individual, and hemorrhagic vs. non-hemorrhagic nature. (From Adams et al., Principles of Neurology, 6th ed, pp777-810)	2.01	1	0
Neoplasms, Pleural [description not available]	2.03	1	0
Calcification, Pathologic [description not available]	2.38	2	0
Arteriosclerosis Thickening and loss of elasticity of the walls of ARTERIES of all sizes. There are many forms classified by the types of lesions and arteries involved, such as ATHEROSCLEROSIS with fatty lesions in the ARTERIAL INTIMA of medium and large muscular arteries.	2.37	2	0
Calcinosis Pathologic deposition of calcium salts in tissues.	2.38	2	0
Cirrhoses, Experimental Liver [description not available]	1.96	1	0
Obstructive Lung Diseases [description not available]	2.91	4	0
Lung Diseases, Obstructive Any disorder marked by obstruction of conducting airways of the lung. AIRWAY OBSTRUCTION may be acute, chronic, intermittent, or persistent.	2.91	4	0
Sclerosis, Systemic [description not available]	1.98	1	0
Scleroderma, Systemic A chronic multi-system disorder of CONNECTIVE TISSUE. It is characterized by SCLEROSIS in the SKIN, the LUNGS, the HEART, the GASTROINTESTINAL TRACT, the KIDNEYS, and the MUSCULOSKELETAL SYSTEM. Other important features include diseased small BLOOD VESSELS and AUTOANTIBODIES. The disorder is named for its most prominent feature (hard skin), and classified into subsets by the extent of skin thickening: LIMITED SCLERODERMA and DIFFUSE SCLERODERMA.	1.98	1	0
Bronchiectasis Persistent abnormal dilatation of the bronchi.	2.4	2	0
ALS - Amyotrophic Lateral Sclerosis [description not available]	1.98	1	0
Dermatoses [description not available]	2.68	3	0
Amyotrophic Lateral Sclerosis A degenerative disorder affecting upper MOTOR NEURONS in the brain and lower motor neurons in the brain stem and SPINAL CORD. Disease onset is usually after the age of 50 and the process is usually fatal within 3 to 6 years. Clinical manifestations include progressive weakness, atrophy, FASCICULATION, hyperreflexia, DYSARTHRIA, dysphagia, and eventual paralysis of respiratory function. Pathologic features include the replacement of motor neurons with fibrous ASTROCYTES and atrophy of anterior SPINAL NERVE ROOTS and corticospinal tracts. (From Adams et al., Principles of Neurology, 6th ed, pp1089-94)	1.98	1	0
Skin Diseases Diseases involving the DERMIS or EPIDERMIS.	2.68	3	0
Diabetes Mellitus A heterogeneous group of disorders characterized by HYPERGLYCEMIA and GLUCOSE INTOLERANCE.	1.98	1	0
Varices [description not available]	1.99	1	0
Varicose Veins Enlarged and tortuous VEINS.	1.99	1	0
Left Ventricular Hypertrophy [description not available]	1.99	1	0
Hypertrophy, Left Ventricular Enlargement of the LEFT VENTRICLE of the heart. This increase in ventricular mass is attributed to sustained abnormal pressure or volume loads and is a contributor to cardiovascular morbidity and mortality.	1.99	1	0
Esophago-Pharyngeal Diverticula [description not available]	1.99	1	0
Cirrhosis, Liver [description not available]	2.92	1	0
Bilharziasis [description not available]	2.92	1	0
Liver Cirrhosis Liver disease in which the normal microcirculation, the gross vascular anatomy, and the hepatic architecture have been variably destroyed and altered with fibrous septa surrounding regenerated or regenerating parenchymal nodules.	2.92	1	0
Schistosomiasis Infection with flukes (trematodes) of the genus SCHISTOSOMA. Three species produce the most frequent clinical diseases: SCHISTOSOMA HAEMATOBIUM (endemic in Africa and the Middle East), SCHISTOSOMA MANSONI (in Egypt, northern and southern Africa, some West Indies islands, northern 2/3 of South America), and SCHISTOSOMA JAPONICUM (in Japan, China, the Philippines, Celebes, Thailand, Laos). S. mansoni is often seen in Puerto Ricans living in the United States.	2.92	1	0
Dilatation, Pathologic The condition of an anatomical structure's being dilated beyond normal dimensions.	2	1	0
Marfan Syndrome, Type I [description not available]	2	1	0
Marfan Syndrome An autosomal dominant disorder of CONNECTIVE TISSUE with abnormal features in the heart, the eye, and the skeleton. Cardiovascular manifestations include MITRAL VALVE PROLAPSE, dilation of the AORTA, and aortic dissection. Other features include lens displacement (ectopia lentis), disproportioned long limbs and enlarged DURA MATER (dural ectasia). Marfan syndrome (type 1) is associated with mutations in the gene encoding FIBRILLIN-1 (FBN1), a major element of extracellular microfibrils of connective tissue. Mutations in the gene encoding TYPE II TGF-BETA RECEPTOR (TGFBR2) are associated with Marfan syndrome type 2.	2	1	0
Cell Transformation, Neoplastic Cell changes manifested by escape from control mechanisms, increased growth potential, alterations in the cell surface, karyotypic abnormalities, morphological and biochemical deviations from the norm, and other attributes conferring the ability to invade, metastasize, and kill.	1.95	1	0
Vascular Diseases Pathological processes involving any of the BLOOD VESSELS in the cardiac or peripheral circulation. They include diseases of ARTERIES; VEINS; and rest of the vasculature system in the body.	1.95	1	0
Aneurysm, Aortic [description not available]	1.98	1	0
Aortic Aneurysm An abnormal balloon- or sac-like dilatation in the wall of AORTA.	1.98	1	0
Cutis Laxa A group of connective tissue diseases in which skin hangs in loose pendulous folds. It is believed to be associated with decreased elastic tissue formation as well as an abnormality in elastin formation. Cutis laxa is usually a genetic disease, but acquired cases have been reported. (From Dorland, 27th ed)	1.97	1	0
Groenblad-Strandberg Syndrome [description not available]	1.97	1	0
Pseudoxanthoma Elasticum An inherited disorder of connective tissue with extensive degeneration and calcification of ELASTIC TISSUE primarily in the skin, eye, and vasculature. At least two forms exist, autosomal recessive and autosomal dominant. This disorder is caused by mutations of one of the ATP-BINDING CASSETTE TRANSPORTERS. Patients are predisposed to MYOCARDIAL INFARCTION and GASTROINTESTINAL HEMORRHAGE.	1.97	1	0
Chronic Lung Injury [description not available]	1.97	1	0
Aneurysm, Arteriovenous [description not available]	1.97	1	0
Fibromatosis [description not available]	1.96	1	0
Cancer of Skin [description not available]	1.96	1	0
Fibroma A benign tumor of fibrous or fully developed connective tissue.	1.96	1	0
Skin Neoplasms Tumors or cancer of the SKIN.	1.96	1	0
Infant, Premature, Diseases Diseases that occur in PREMATURE INFANTS.	1.96	1	0
Bronchopulmonary Dysplasia A chronic lung disease developed after OXYGEN INHALATION THERAPY or mechanical ventilation (VENTILATION, MECHANICAL) usually occurring in certain premature infants (INFANT, PREMATURE) or newborn infants with respiratory distress syndrome (RESPIRATORY DISTRESS SYNDROME, NEWBORN). Histologically, it is characterized by the unusual abnormalities of the bronchioles, such as METAPLASIA, decrease in alveolar number, and formation of CYSTS.	1.96	1	0

isodesmosine

Description

Cross-References

Synonyms (16)

Research Excerpts

Toxicity

Roles (1)

Drug Classes (2)

Research

Studies (149)

Market Indicators

Research Demand Index: 42.18

Study Types