cystic fibrosis transmembrane conductance regulator (505-511) profile

cystic fibrosis transmembrane conductance regulator (505-511): synthetic peptide deduced from the sequence corresponding to positions 505 to 511 of cystic fibrosis transmembrane conductance regulator [Medical Subject Headings (MeSH), National Library of Medicine, extracted Dec-2023]

ID Source	ID
PubMed CID	132758
MeSH ID	M0200405

Synonym
146289-28-3
cystic fibrosis transmembrane conductance regulator peptide (505-511)
l-serine, n-(n-(n-(n-(n-l-asparaginyl-l-isoleucyl)-l-isoleucyl)-l-phenylalanyl)glycyl)-l-valyl-
cftr (505-511)
cystic fibrosis transmembrane conductance regulator (505-511)
(2s)-2-[[(2s)-2-[[2-[[(2s)-2-[[(2s,3s)-2-[[(2s,3s)-2-[[(2s)-2,4-diamino-4-oxobutanoyl]amino]-3-methylpentanoyl]amino]-3-methylpentanoyl]amino]-3-phenylpropanoyl]amino]acetyl]amino]-3-methylbutanoyl]amino]-3-hydroxypropanoic acid
n-[17-amino-8-benzyl-11,14-di(butan-2-yl)-1,4,7,10,13,16,19-heptahydroxy-19-imino-2-(propan-2-yl)-3,6,9,12,15-pentaazanonadeca-3,6,9,12,15-pentaen-1-ylidene]serine
DTXSID90932806

Excerpt	Relevance	Reference
" Efficacy was determined using nasal transepithelial potential difference and sweat chloride measurements prior to dosing and at 1, 2, and 4 hr postdose."	( A phase I randomized, multicenter trial of CPX in adult subjects with mild cystic fibrosis. Ahrens, RC; Aitken, ML; Hilliard, KA; Kelley, TJ; Konstan, MW; Launspach, J; McCarty, NA; Milgram, LJ; Regelmann, WE; Standaert, TA; Teresi, M; Tuthill, C; Weatherly, MR, 2002)	0.31

Timeframe	Studies, This Drug (%)	All Drugs %
pre-1990	0 (0.00)	18.7374
1990's	1 (5.88)	18.2507
2000's	12 (70.59)	29.6817
2010's	4 (23.53)	24.3611
2020's	0 (0.00)	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	1 (5.88%)	5.53%
Reviews	3 (17.65%)	6.00%
Case Studies	3 (17.65%)	4.05%
Observational	0 (0.00%)	0.25%
Other	10 (58.82%)	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
chlorine chloride : A halide anion formed when chlorine picks up an electron to form an an anion.	4.3	4	1	halide anion; monoatomic chlorine	cofactor; Escherichia coli metabolite; human metabolite
1,3-dipropyl-8-cyclopentylxanthine DPCPX : An oxopurine that is 7H-xanthine substituted at positions 1 and 3 by propyl groups and at position 8 by a cyclohexyl group.	3.39	1	1	oxopurine	adenosine A1 receptor antagonist; EC 3.1.4.* (phosphoric diester hydrolase) inhibitor
glyburide Glyburide: An antidiabetic sulfonylurea derivative with actions like those of chlorpropamide. glyburide : An N-sulfonylurea that is acetohexamide in which the acetyl group is replaced by a 2-(5-chloro-2-methoxybenzamido)ethyl group.	2.01	1	0	monochlorobenzenes; N-sulfonylurea	anti-arrhythmia drug; EC 2.7.1.33 (pantothenate kinase) inhibitor; EC 3.6.3.49 (channel-conductance-controlling ATPase) inhibitor; hypoglycemic agent
1-methyl-3-isobutylxanthine 1-Methyl-3-isobutylxanthine: A potent cyclic nucleotide phosphodiesterase inhibitor; due to this action, the compound increases cyclic AMP and cyclic GMP in tissue and thereby activates CYCLIC NUCLEOTIDE-REGULATED PROTEIN KINASES. 3-isobutyl-1-methylxanthine : An oxopurine that is xanthine which is substituted at positions 1 and 3 by methyl and isobutyl groups, respectively.	2.01	1	0	3-isobutyl-1-methylxanthine
fenamic acid fenamic acid: has chloride and potassium channel-blocking activity; RN given refers to parent cpd. fenamic acid : An aminobenzoic acid that is the N-phenyl derivative of anthranilic acid. It acts as a parent skeleton for the synthesis of several non-steroidal anti-inflammatory drugs.	2.01	1	0	aminobenzoic acid; secondary amino compound	membrane transport modulator
5-nitro-2-(3-phenylpropylamino)benzoic acid 5-nitro-2-(3-phenylpropylamino)benzoic acid: structure given in first source; chloride channel antagonist	2.01	1	0	nitrobenzoic acid
carbostyril Quinolones: A group of derivatives of naphthyridine carboxylic acid, quinoline carboxylic acid, or NALIDIXIC ACID.. quinolin-2(1H)-one : A quinolone that is 1,2-dihydroquinoline substituted by an oxo group at position 2.	2.51	2	0	monohydroxyquinoline; quinolone	bacterial xenobiotic metabolite
uridine triphosphate Uridine Triphosphate: Uridine 5'-(tetrahydrogen triphosphate). A uracil nucleotide containing three phosphate groups esterified to the sugar moiety.	2.01	1	0	pyrimidine ribonucleoside 5'-triphosphate; uridine 5'-phosphate	Escherichia coli metabolite; mouse metabolite
alpha-aminopyridine alpha-aminopyridine: RN given refers to parent cpd; structure in Merck Index, 9th ed, #485. aminopyridine : Compounds containing a pyridine skeleton substituted by one or more amine groups.	2.51	2	0
amiloride Amiloride: A pyrazine compound inhibiting SODIUM reabsorption through SODIUM CHANNELS in renal EPITHELIAL CELLS. This inhibition creates a negative potential in the luminal membranes of principal cells, located in the distal convoluted tubule and collecting duct. Negative potential reduces secretion of potassium and hydrogen ions. Amiloride is used in conjunction with DIURETICS to spare POTASSIUM loss. (From Gilman et al., Goodman and Gilman's The Pharmacological Basis of Therapeutics, 9th ed, p705). amiloride : A member of the class of pyrazines resulting from the formal monoacylation of guanidine with the carboxy group of 3,5-diamino-6-chloropyrazine-2-carboxylic acid.	2.01	1	0	aromatic amine; guanidines; organochlorine compound; pyrazines	diuretic; sodium channel blocker
tobramycin Tobramycin: An aminoglycoside, broad-spectrum antibiotic produced by Streptomyces tenebrarius. It is effective against gram-negative bacteria, especially the PSEUDOMONAS species. It is a 10% component of the antibiotic complex, NEBRAMYCIN, produced by the same species.. tobramycin : A amino cyclitol glycoside that is kanamycin B lacking the 3-hydroxy substituent from the 2,6-diaminoglucose ring.	2.01	1	0	amino cyclitol glycoside	antibacterial agent; antimicrobial agent; toxin
stilbenes Stilbenes: Organic compounds that contain 1,2-diphenylethylene as a functional group.. trans-stilbene : The trans-isomer of stilbene.	2.01	1	0	stilbene
4-acetamido-4'-isothiocyanatostilbene-2,2'-disulfonic acid 4-Acetamido-4'-isothiocyanatostilbene-2,2'-disulfonic Acid: A non-penetrating amino reagent (commonly called SITS) which acts as an inhibitor of anion transport in erythrocytes and other cells.	2.01	1	0	stilbenoid
4,4'-dinitro-2,2'-stilbenedisulfonic acid 4,4'-dinitro-2,2'-stilbenedisulfonic acid: RN given refers to parent cpd without isomeric designation	2.01	1	0	4,4'-dinitrostilbene-2,2'-disulfonic acid
4,4'-diisothiocyanostilbene-2,2'-disulfonic acid 4,4'-Diisothiocyanostilbene-2,2'-Disulfonic Acid: An inhibitor of anion conductance including band 3-mediated anion transport.	2.01	1	0
vx-770 ivacaftor: a CFTR potentiator; structure in first source. ivacaftor : An aromatic amide obtained by formal condensation of the carboxy group of 4-oxo-1,4-dihydroquinoline-3-carboxylic acid with the amino group of 5-amino-2,4-di-tert-butylphenol. Used for the treatment of cystic fibrosis.	2.51	2	0	aromatic amide; monocarboxylic acid amide; phenols; quinolone	CFTR potentiator; orphan drug
lumacaftor lumacaftor: a corrector of CF transmembrane conductance regulator (CTFR); structure in first source. lumacaftor : An aromatic amide obtained by formal condensation of the carboxy group of 1-(2,2-difluoro-1,3-benzodioxol-5-yl)cyclopropane-1-carboxylic acid with the aromatic amino group of 3-(6-amino-3-methylpyridin-2-yl)benzoic acid. Used for the treatment of cystic fibrosis.	2.51	2	0	aromatic amide; benzodioxoles; benzoic acids; cyclopropanes; organofluorine compound; pyridines	CFTR potentiator; orphan drug
cyclic gmp Cyclic GMP: Guanosine cyclic 3',5'-(hydrogen phosphate). A guanine nucleotide containing one phosphate group which is esterified to the sugar moiety in both the 3'- and 5'-positions. It is a cellular regulatory agent and has been described as a second messenger. Its levels increase in response to a variety of hormones, including acetylcholine, insulin, and oxytocin and it has been found to activate specific protein kinases. (From Merck Index, 11th ed). 3',5'-cyclic GMP : A 3',5'-cyclic purine nucleotide in which the purine nucleobase is specified as guanidine.	2.01	1	0	3',5'-cyclic purine nucleotide; guanyl ribonucleotide	Escherichia coli metabolite; human metabolite; mouse metabolite; plant metabolite; Saccharomyces cerevisiae metabolite
trypsinogen Trypsinogen: The inactive proenzyme of trypsin secreted by the pancreas, activated in the duodenum via cleavage by enteropeptidase. (Stedman, 25th ed)	3.11	1	0

Condition	Relationship Strength	Studies	Trials
Cystic Fibrosis of Pancreas [description not available]	6.81	14	1
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.	6.81	14	1
Malnourishment [description not available]	2.05	1	0
Malnutrition An imbalanced nutritional status resulting from insufficient intake of nutrients to meet normal physiological requirement.	2.05	1	0
ALS - Amyotrophic Lateral Sclerosis [description not available]	2.08	1	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)	2.08	1	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)	2.01	1	0
Genetic Predisposition [description not available]	2.93	1	0
Acute Edematous Pancreatitis [description not available]	2.93	1	0
Pancreatitis INFLAMMATION of the PANCREAS. Pancreatitis is classified as acute unless there are computed tomographic or endoscopic retrograde cholangiopancreatographic findings of CHRONIC PANCREATITIS (International Symposium on Acute Pancreatitis, Atlanta, 1992). The two most common forms of acute pancreatitis are ALCOHOLIC PANCREATITIS and gallstone pancreatitis.	2.93	1	0
Body Weight The mass or quantity of heaviness of an individual. It is expressed by units of pounds or kilograms.	2.01	1	0
Stunted Growth [description not available]	2.01	1	0
Growth Disorders Deviations from the average values for a specific age and sex in any or all of the following: height, weight, skeletal proportions, osseous development, or maturation of features. Included here are both acceleration and retardation of growth.	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.	2.42	2	0
Disease, Pulmonary [description not available]	2.95	1	0
Lung Diseases Pathological processes involving any part of the LUNG.	2.95	1	0

cystic fibrosis transmembrane conductance regulator (505-511)

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