Page last updated: 2024-09-28

alpha-Chain Disease

A condition that is caused by HYPERPLASIA of LYMPHOCYTES in the small intestine (INTESTINE, SMALL) and the mesenteric LYMPH NODES. These lymphocytes produce an anomalous alpha heavy chain protein. Generally, these IPSID patients have either concurrent LYMPHOMA or develop lymphoma within a few years. The disease was first described in the Mediterranean region and is characterized by malabsorption; WEIGHT LOSS; DIARRHEA; and STEATORRHEA.

Synonyms(5)

Synonym
IPSID
Immunoproliferative Small Intestinal Disease
Lymphoma, Mediterranean
Heavy Chain Disease, IgA Type
alpha-Chain Disease

Research Excerpts

Context

ExcerptReference
"IPSID has been described mainly in the Mediterranean, Middle East, and African countries."( Economidou, I; Manousos, ON; Papadakis, T; Triantafillidis, JK; Vaslamatzis, MM; Zafiropoulou, R, 2006)

Treatment

ExcerptReference
"We report a case of Mediterranean lymphoma treated with antibiotics."( Asahara, M; Chiba, T; Fujimori, T; Fukuda, H; Kawanami, C; Kinoshita, Y; Matsumoto, S; Matsushima, Y; Waki, S, 1996)

Research

Studies (27)

TimeframeStudies, This Condition (%)All Conditions %
pre-19906 (22.22)23.3326
1990's14 (51.85)12.5806
2000's5 (18.52)18.1394
2010's2 (7.41)28.8240
2020's0 (0.00)9.53
DrugIndicatedRelationship StrengthStudiesTrials
lansoprazole0low10
mebendazole0low10
metronidazole0low30
mitoxantrone0low10
omeprazole0low40
pantoprazole0low10
procarbazine0medium21
tinidazole0low10
prednisolone0low20
prednisone0medium101
amoxicillin0low40
clarithromycin0low20
methotrexate0low10
bismuth tripotassium dicitrate0low10
arginine0low10
tetracycline0medium91

Protein Targets (477)

ProteinPotency MeasurementsInhibition MeasurementsActivation MeasurementsDrugs
thyroid stimulating hormone receptor3003
cytochrome P450 2D6 isoform 13003
geminin7007
lethal factor (plasmid)2002
lamin isoform A-delta109009
Bile salt export pump011011
Major prion protein0011
Trypanothione reductase0101
Chain A, MAJOR APURINIC/APYRIMIDINIC ENDONUCLEASE3003
Chain A, Beta-lactamase2002
Chain A, 2-oxoglutarate Oxygenase6006
Beta-lactamase 0001
Beta-lactamase 0001
Beta-lactamase 0001
Beta-lactamase 0001
AR protein8008
apical membrane antigen 1, AMA14004
estrogen nuclear receptor alpha110011
Beta-lactamase 0001
peptidyl-prolyl cis-trans isomerase NIMA-interacting 12002
DNA polymerase eta isoform 13003
DNA polymerase iota isoform a (long)4004
urokinase-type plasminogen activator precursor2002
plasminogen precursor2002
urokinase plasminogen activator surface receptor precursor2002
DNA polymerase kappa isoform 13003
ATP-binding cassette sub-family C member 3010010
Multidrug resistance-associated protein 4010011
Bile salt export pump0202
Beta-lactamase SHV-10001
Beta-lactamase SHV-10001
Cytochrome P450 2C9 0505
Beta-lactamase0001
Kappa-type opioid receptor0102
Solute carrier family 15 member 10101
Solute carrier family 15 member 10101
Cytochrome P450 2J20404
B2 metallo-beta-lactamase 0001
Solute carrier family 15 member 20101
Beta-lactamase 0001
Beta-lactamase 0001
Solute carrier family 15 member 20101
Beta-lactamase 0001
Beta-lactamase 0001
Canalicular multispecific organic anion transporter 1010010
GLI family zinc finger 36006
cytochrome P450 family 3 subfamily A polypeptide 46006
estrogen-related nuclear receptor alpha100010
thyroid hormone receptor beta isoform 26006
importin subunit beta-1 isoform 13003
mitogen-activated protein kinase 13003
snurportin-13003
GTP-binding nuclear protein Ran isoform 12002
streptokinase A precursor0011
ATP-dependent translocase ABCB10505
Cytochrome P450 3A40202
Potassium voltage-gated channel subfamily H member 20404
Solute carrier organic anion transporter family member 1B30304
Solute carrier organic anion transporter family member 1B10101
Chain A, TYROSYL-DNA PHOSPHODIESTERASE4004
Chain A, Putative fructose-1,6-bisphosphate aldolase2002
Chain A, HADH2 protein4004
Chain B, HADH2 protein4004
Chain A, JmjC domain-containing histone demethylation protein 3A3003
Luciferase3003
thioredoxin reductase2002
15-lipoxygenase, partial5005
phosphopantetheinyl transferase3003
RAR-related orphan receptor gamma5005
ATAD5 protein, partial3003
USP1 protein, partial5005
GLS protein3003
TDP1 protein9009
Microtubule-associated protein tau4004
aldehyde dehydrogenase 1 family, member A19009
hypoxia-inducible factor 1, alpha subunit (basic helix-loop-helix transcription factor)4004
estrogen receptor 2 (ER beta)5005
nuclear receptor subfamily 1, group I, member 37007
progesterone receptor5005
regulator of G-protein signaling 43003
EWS/FLI fusion protein9009
retinoic acid nuclear receptor alpha variant 15005
retinoid X nuclear receptor alpha4004
pregnane X nuclear receptor8008
G4004
cytochrome P450 2D62002
Parkin2002
bromodomain adjacent to zinc finger domain 2B2002
peroxisome proliferator activated receptor gamma4004
vitamin D (1,25- dihydroxyvitamin D3) receptor5005
alpha-galactosidase2002
euchromatic histone-lysine N-methyltransferase 24004
aryl hydrocarbon receptor5005
cytochrome P450, family 19, subfamily A, polypeptide 1, isoform CRA_a4004
nuclear receptor subfamily 1, group I, member 24004
Replicase polyprotein 1ab0101
lysosomal alpha-glucosidase preproprotein1001
NPC intracellular cholesterol transporter 1 precursor2002
peripheral myelin protein 22 isoform 11001
cytochrome P450 2C19 precursor3003
cytochrome P450 2C9 precursor2002
15-hydroxyprostaglandin dehydrogenase [NAD(+)] isoform 14004
atrial natriuretic peptide receptor 1 precursor2002
vitamin D3 receptor isoform VDRA7007
chromobox protein homolog 15005
phosphoethanolamine/phosphocholine phosphatase isoform 10101
heat shock protein beta-12002
atrial natriuretic peptide receptor 2 precursor2002
ras-related protein Rab-9A2002
serine/threonine-protein kinase PLK12002
nuclear factor erythroid 2-related factor 2 isoform 16006
nuclear receptor ROR-gamma isoform 13003
peripheral myelin protein 224004
cytochrome P450 3A4 isoform 15005
M-phase phosphoprotein 83003
muscarinic acetylcholine receptor M12002
Gamma-aminobutyric acid receptor subunit pi5005
Polyunsaturated fatty acid lipoxygenase ALOX15B3003
Voltage-dependent calcium channel gamma-2 subunit4004
N(G),N(G)-dimethylarginine dimethylaminohydrolase 10303
Interferon beta6006
HLA class I histocompatibility antigen, B alpha chain 4004
Sodium/potassium-transporting ATPase subunit alpha-1 0303
Sodium/potassium-transporting ATPase subunit beta-10303
Amyloid-beta precursor protein2002
Neuronal acetylcholine receptor subunit alpha-41001
Replicase polyprotein 1ab0101
Cytochrome P450 2D60202
Microtubule-associated protein tau0101
Neuronal acetylcholine receptor subunit beta-21001
Sodium/potassium-transporting ATPase subunit alpha-30303
Sodium/potassium-transporting ATPase subunit beta-20303
Gamma-aminobutyric acid receptor subunit beta-15005
Gamma-aminobutyric acid receptor subunit delta5005
Gamma-aminobutyric acid receptor subunit gamma-25005
Glutamate receptor 24004
Gamma-aminobutyric acid receptor subunit alpha-55005
Gamma-aminobutyric acid receptor subunit alpha-35005
Gamma-aminobutyric acid receptor subunit gamma-15005
Gamma-aminobutyric acid receptor subunit alpha-25005
Gamma-aminobutyric acid receptor subunit alpha-45005
Gamma-aminobutyric acid receptor subunit gamma-35005
Indoleamine 2,3-dioxygenase 10202
Gamma-aminobutyric acid receptor subunit alpha-65005
Cytochrome P450 2C190203
Alpha-synuclein1102
Histamine H2 receptor4004
Phosphatidylinositol 5-phosphate 4-kinase type-2 alpha1001
Fatty acid synthase0606
Sodium/potassium-transporting ATPase subunit alpha-20303
Sodium/potassium-transporting ATPase subunit beta-30303
Sodium/potassium-transporting ATPase subunit gamma0303
WD repeat-containing protein 50202
Gamma-aminobutyric acid receptor subunit alpha-15005
Gamma-aminobutyric acid receptor subunit beta-35005
Gamma-aminobutyric acid receptor subunit beta-25005
Histone-lysine N-methyltransferase 2A0202
Sodium/potassium-transporting ATPase subunit alpha-40303
Cytosolic endo-beta-N-acetylglucosaminidase0202
Indoleamine 2,3-dioxygenase 20202
GABA theta subunit5005
Inositol hexakisphosphate kinase 14004
Ataxin-25005
Gamma-aminobutyric acid receptor subunit epsilon5005
Broad substrate specificity ATP-binding cassette transporter ABCG20406
cytochrome P450 2C9, partial4004
ATP-dependent phosphofructokinase3003
SMAD family member 22002
Fumarate hydratase2002
GALC protein2002
PPM1D protein2002
SMAD family member 32002
glucocorticoid receptor [Homo sapiens]4004
farnesoid X nuclear receptor3003
polyprotein2002
v-jun sarcoma virus 17 oncogene homolog (avian)4004
Histone H2A.x4004
histone deacetylase 9 isoform 31001
huntingtin isoform 23003
ubiquitin carboxyl-terminal hydrolase 2 isoform a2002
Tyrosine-protein kinase ABL10213
Cellular tumor antigen p536006
Methionine aminopeptidase0101
Proto-oncogene tyrosine-protein kinase Src0202
Vascular endothelial growth factor receptor 20101
Spike glycoprotein4004
Disintegrin and metalloproteinase domain-containing protein 171001
Fatty-acid amide hydrolase 10101
Inositol monophosphatase 12002
ATPase family AAA domain-containing protein 53003
Calmodulin-domain protein kinase 10101
Chain B, DIHYDROFOLATE REDUCTASE0011
Chain B, DIHYDROFOLATE REDUCTASE0011
Chain B, DIHYDROFOLATE REDUCTASE0011
Chain A, Ferritin light chain2002
acid sphingomyelinase1001
dopamine D1 receptor2002
ClpP2002
67.9K protein2002
peroxisome proliferator-activated receptor delta2002
Thymidylate synthase 0101
IDH12002
Thymidylate synthase 0101
Bloom syndrome protein isoform 12002
cellular tumor antigen p53 isoform a2002
polyunsaturated fatty acid lipoxygenase ALOX121001
nuclear factor erythroid 2-related factor 2 isoform 24004
transcriptional regulator ERG isoform 31001
DNA polymerase beta1001
nuclear receptor coactivator 1 isoform 1 [Homo sapiens]0101
flap endonuclease 12002
transactivating tegument protein VP16 [Human herpesvirus 1]0101
survival motor neuron protein isoform d4004
muscleblind-like protein 1 isoform 14004
histone acetyltransferase KAT2A isoform 14004
caspase-1 isoform alpha precursor1001
nuclear receptor coactivator 3 isoform a0101
Toll-like receptor 40101
Fatty-acid amide hydrolase 10101
ATP-binding cassette sub-family C member 30102
Dihydrofolate reductase0203
Dihydrofolate reductase0203
Dihydrofolate reductase 0101
Dihydrofolate reductase0101
Dihydrofolate reductase0101
Dihydrofolate reductase0203
Thymidylate synthase0101
Carbonic anhydrase 20011
Thymidylate synthase0202
Integrin beta-32002
Bifunctional dihydrofolate reductase-thymidylate synthase0101
Thymidylate synthase0202
Integrin alpha-IIb2002
5-hydroxytryptamine receptor 2C0102
High mobility group protein B10011
Dihydrofolate reductase0101
Thymidylate synthase0101
Dihydrofolate reductase0224
Glucose-6-phosphate 1-dehydrogenase0101
Bifunctional dihydrofolate reductase-thymidylate synthase0101
Folate receptor beta0101
5-hydroxytryptamine receptor 2A0101
Cytochrome P450 11B1, mitochondrial 0101
Folate receptor alpha0101
Dihydrofolate reductase0202
Histidine decarboxylase0112
Trifunctional purine biosynthetic protein adenosine-30101
Cyclin-dependent kinase 20011
Bifunctional purine biosynthesis protein ATIC0202
Multidrug resistance-associated protein 1 0102
Delta-type opioid receptor0213
Mu-type opioid receptor0213
Kappa-type opioid receptor0213
Reduced folate transporter0202
Folylpolyglutamate synthase, mitochondrial0103
6-phosphogluconate dehydrogenase, decarboxylating0101
Caspase-71001
Solute carrier organic anion transporter family member 1A30001
Dihydrofolate reductase0101
Pteridine reductase 10101
Folylpolyglutamate synthase, mitochondrial0103
Bifunctional dihydrofolate reductase-thymidylate synthase0101
TAR DNA-binding protein 433104
Bifunctional dihydrofolate reductase-thymidylate synthase0101
Solute carrier family 22 member 60001
Canalicular multispecific organic anion transporter 10102
Trifunctional purine biosynthetic protein adenosine-30101
Solute carrier family 22 member 200101
Dihydrofolate reductase 0101
Solute carrier family 22 member 80002
Solute carrier family 22 member 60101
Dihydrofolate reductase0101
ATP-binding cassette sub-family C member 110001
Proton-coupled folate transporter0101
Multidrug resistance associated protein0102
Solute carrier family 22 member 110002
Protein-arginine deiminase type-40202
Thioredoxin reductase 0101
thyroid hormone receptor beta isoform a1001
Flavodoxin0001
Voltage-dependent L-type calcium channel subunit alpha-1F0303
Voltage-dependent L-type calcium channel subunit alpha-1D 0303
Voltage-dependent L-type calcium channel subunit alpha-1S0303
Voltage-dependent L-type calcium channel subunit alpha-1C0303
Lanosterol 14-alpha demethylase0101
Chain A, Probable serine/threonine-protein kinase pknB0101
Chain A, ATP-DEPENDENT DNA HELICASE Q11001
glp-1 receptor, partial1001
WRN1001
NFKB1 protein, partial1001
Thrombopoietin1001
Smad31001
DNA polymerase III, partial1001
hypothetical protein, conserved1001
nonstructural protein 10101
LANA0001
arylsulfatase A1001
pyruvate kinase1001
heat shock 70kDa protein 5 (glucose-regulated protein, 78kDa)1001
phospholipase A2, group III0101
interferon gamma precursor0001
histone-lysine N-methyltransferase NSD2 isoform 10001
protein AF-9 isoform a0001
serine/threonine-protein kinase mTOR isoform 11001
tyrosine-protein kinase Yes1001
histone-lysine N-methyltransferase 2A isoform 2 precursor1001
dual specificity tyrosine-phosphorylation-regulated kinase 1A0001
rac GTPase-activating protein 1 isoform a0101
Vpr1001
DNA repair protein RAD52 homolog isoform a0001
pyruvate kinase PKM isoform b1001
Solute carrier family 22 member 20202
Solute carrier family 22 member 1 0303
5-hydroxytryptamine receptor 40202
Solute carrier family 22 member 30202
Epidermal growth factor receptor0101
Glycoprotein hormones alpha chain1001
Receptor tyrosine-protein kinase erbB-20101
Tyrosine-protein kinase Fyn0101
Aldo-keto reductase family 1 member B10202
Muscarinic acetylcholine receptor M20303
Muscarinic acetylcholine receptor M40202
Proto-oncogene tyrosine-protein kinase ROS0101
Ornithine decarboxylase1001
Muscarinic acetylcholine receptor M10202
DNA topoisomerase 2-alpha0102
Polyunsaturated fatty acid lipoxygenase ALOX150101
Angiotensin-converting enzyme0202
DNA topoisomerase 20001
Casein kinase II subunit alpha'0101
Sodium-dependent noradrenaline transporter 0202
5-hydroxytryptamine receptor 2C0202
Guanine nucleotide-binding protein G1001
Casein kinase II subunit beta0101
Casein kinase II subunit alpha0101
DNA topoisomerase 2-beta0001
Tyrosine-protein kinase BTK0101
NEDD8-activating enzyme E1 regulatory subunit0011
Nuclear receptor subfamily 3 group C member 3 0202
Integrase 0202
Multidrug and toxin extrusion protein 20202
Casein kinase II subunit alpha 30101
NEDD8-activating enzyme E1 catalytic subunit0011
Multidrug and toxin extrusion protein 10202
Single-stranded DNA cytosine deaminase1001
P2Y purinoceptor 120202
phosphoglycerate kinase1001
2,3-bisphosphoglycerate-independent phosphoglycerate mutase1001
acetylcholinesterase2002
Mcl-10101
hypoxia-inducible factor 1 alpha subunit2002
activating transcription factor 61001
nuclear factor of kappa light polypeptide gene enhancer in B-cells 1 (p105), isoform CRA_a1001
pyruvate kinase PKM isoform a1001
cGMP-specific 3',5'-cyclic phosphodiesterase0101
Bifunctional cytochrome P450/NADPH--P450 reductase0011
UDP-glucuronosyltransferase 2B70101
Potassium-transporting ATPase subunit beta0101
Potassium-transporting ATPase alpha chain 10101
UDP-glucuronosyltransferase 1-60101
Potassium-transporting ATPase alpha chain 10101
UDP-glucuronosyltransferase 1A1 0101
UDP-glucuronosyltransferase 1A40101
Bombesin receptor subtype-30011
UDP-glucuronosyltransferase 2B10 0101
Potassium-transporting ATPase subunit beta0101
Potassium-transporting ATPase alpha chain 20001
Glutathione S-transferase omega-10101
Muscarinic acetylcholine receptor0101
Delta-type opioid receptor0101
Mu-type opioid receptor0112
Delta-type opioid receptor0101
Kappa-type opioid receptor0101
Mu-type opioid receptor0101
Mu-type opioid receptor0101
Chain A, Cruzipain1001
potassium voltage-gated channel subfamily H member 2 isoform d2002
Gamma-aminobutyric acid receptor subunit alpha-10101
Gamma-aminobutyric acid receptor subunit gamma-20011
Gamma-aminobutyric acid receptor subunit beta-30101
Gamma-aminobutyric acid receptor subunit alpha-20011
Gamma-aminobutyric acid receptor subunit beta-20011
luciferase0001
Fatty acid-binding protein, liver0101
Estrogen receptor0202
Glucocorticoid receptor04211
Progesterone receptor0203
Glucocorticoid receptor0112
Glucocorticoid receptor0011
Glycine receptor subunit alpha-10404
Corticosteroid-binding globulin0101
Mineralocorticoid receptor 0213
Androgen receptor0202
Glutamine synthetase0011
Androgen receptor0101
Glycine receptor subunit beta0404
Glycine receptor subunit alpha-20404
Glycine receptor subunit alpha-30404
Histamine H1 receptor0011
Prostaglandin G/H synthase 20101
Nociceptin receptor0011
Estrogen receptor beta0202
pregnane X receptor1001
thioredoxin glutathione reductase1001
Nuclear receptor ROR-gamma1001
90-kda heat shock protein beta HSP90 beta, partial0101
estrogen receptor beta isoform 10101
heat shock protein HSP 90-alpha isoform 20101
tyrosine-protein phosphatase non-receptor type 7 isoform 20101
Dihydroxyacetone phosphate acyltransferase0001
30S ribosomal protein S60101
30S ribosomal protein S70101
50S ribosomal protein L150101
Tetracycline resistance protein, class B0001
72 kDa type IV collagenase0101
Stromelysin-10101
50S ribosomal protein L100101
50S ribosomal protein L110101
50S ribosomal protein L7/L120101
50S ribosomal protein L190101
50S ribosomal protein L10101
50S ribosomal protein L200101
50S ribosomal protein L270101
50S ribosomal protein L280101
50S ribosomal protein L290101
50S ribosomal protein L310101
50S ribosomal protein L31 type B0101
50S ribosomal protein L320101
50S ribosomal protein L330101
50S ribosomal protein L340101
50S ribosomal protein L350101
50S ribosomal protein L360101
30S ribosomal protein S100101
30S ribosomal protein S110101
30S ribosomal protein S120101
30S ribosomal protein S130101
30S ribosomal protein S160101
30S ribosomal protein S180101
30S ribosomal protein S190101
30S ribosomal protein S200101
30S ribosomal protein S20101
30S ribosomal protein S30101
30S ribosomal protein S40101
30S ribosomal protein S50101
30S ribosomal protein S80101
30S ribosomal protein S90101
50S ribosomal protein L130101
50S ribosomal protein L140101
50S ribosomal protein L160101
50S ribosomal protein L230101
30S ribosomal protein S150101
Multidrug transporter MdfA0101
50S ribosomal protein L170101
50S ribosomal protein L210101
50S ribosomal protein L300101
50S ribosomal protein L60101
30S ribosomal protein S140101
30S ribosomal protein S170101
30S ribosomal protein S10101
50S ribosomal protein L180101
Alpha-1B adrenergic receptor0101
Neutrophil collagenase0101
Alpha-1D adrenergic receptor0101
Alpha-1A adrenergic receptor0101
Collagenase 30101
Cannabinoid receptor 10101
50S ribosomal protein L20101
50S ribosomal protein L30101
50S ribosomal protein L240101
50S ribosomal protein L40101
50S ribosomal protein L220101
50S ribosomal protein L50101
30S ribosomal protein S210101
50S ribosomal protein L250101
50S ribosomal protein L36 20101
Solute carrier family 22 member 70001
nonstructural protein 11001
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]