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ATP-binding cassette, sub-family C (CFTR/MRP), member 8

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Target not currently curated in GtoImmuPdb

Target id: 2594

Nomenclature: ATP-binding cassette, sub-family C (CFTR/MRP), member 8

Abbreviated Name: SUR1

Systematic Nomenclature: ABCC8

Family: ABCC subfamily

Gene and Protein Information Click here for help
Species TM AA Chromosomal Location Gene Symbol Gene Name Reference
Human 17 1581 11p15.1 ABCC8 ATP binding cassette subfamily C member 8
Mouse - 1588 7 B4 Abcc8 ATP-binding cassette, sub-family C (CFTR/MRP), member 8
Rat 17 1582 1q22 Abcc8 ATP binding cassette subfamily C member 8
Previous and Unofficial Names Click here for help
ABC36 | ATP-binding cassette | ATP-binding cassette, subfamily C (CFTR/MRP), member 8 | HHF1 | HI | MRP8 | PHHI | SUR1 | TNDM2
Database Links Click here for help
ChEMBL Target
DrugBank Target
Ensembl Gene
Entrez Gene
Human Protein Atlas
KEGG Gene
OMIM
Orphanet
Pharos
RefSeq Nucleotide
RefSeq Protein
UniProtKB
Wikipedia

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Inhibitors
Key to terms and symbols View all chemical structures Click column headers to sort
Ligand Sp. Action Value Parameter Reference
repaglinide Small molecule or natural product Approved drug Primary target of this compound Ligand has a PDB structure Hs Inhibition 7.0 pIC50 3
pIC50 7.0 (IC50 1.06x10-7 M) [3]
chlorpropamide Small molecule or natural product Approved drug Hs Inhibition - -
Inhibitor Comments
KATP channels are composed of a small inwardly rectifying K+ channel subunit (Kir6.1 or Kir6.2) plus a sulfonylurea receptor (SUR1, SUR2A or SUR2B) belonging to the ATP-binding cassette superfamily [1]. Repaglinide is a member of the carbamoylmethylbenzoic acid chemical family, which inhibits SUR1 [3].
Immuno Process Associations
Immuno Process:  Cytokine production & signalling
GO Annotations:  Associated to 1 GO processes, IEA only
click arrow to show/hide IEA associations
GO:0032760 positive regulation of tumor necrosis factor production IEA
Clinically-Relevant Mutations and Pathophysiology Click here for help
Disease:  Autosomal dominant hyperinsulinism due to SUR1 deficiency
Synonyms: Hyperinsulinemic hypoglycemia [Disease Ontology: DOID:13317]
Hyperinsulinemic hypoglycemia, familial, 1; HHF1 [OMIM: 256450]
Disease Ontology: DOID:13317
OMIM: 256450
Orphanet: ORPHA276575
Disease:  Autosomal recessive hyperinsulinism due to SUR1 deficiency
Synonyms: Hyperinsulinemic hypoglycemia [Disease Ontology: DOID:13317]
Hyperinsulinemic hypoglycemia, familial, 1; HHF1 [OMIM: 256450]
Disease Ontology: DOID:13317
OMIM: 256450
Orphanet: ORPHA79643
Disease:  Diabetes mellitus, permanent neonatal; PNDM
Synonyms: DEND syndrome [Orphanet: ORPHA79134]
Developmental delay-epilepsy-neonatal diabetes syndrome [Orphanet: ORPHA79134]
Permanent neonatal diabetes mellitus [Orphanet: ORPHA99885]
OMIM: 606176
Orphanet: ORPHA99885, ORPHA79134
Disease:  Diabetes mellitus, transient neonatal, 2
Synonyms: Transient neonatal diabetes mellitus [Orphanet: ORPHA99886]
OMIM: 610374
Orphanet: ORPHA99886
Disease:  Diazoxide-resistant focal hyperinsulinism due to SUR1 deficiency
Synonyms: Hyperinsulinemic hypoglycemia, familial, 1; HHF1 [OMIM: 256450]
OMIM: 256450
Orphanet: ORPHA276598
Drugs: 
Disease:  Hypoglycemia, Leucine-Induced; LIH
OMIM: 240800
Disease:  Maturity-onset diabetes of the young; MODY
Disease Ontology: DOID:0050524
OMIM: 606391
Orphanet: ORPHA552
General Comments
The sulfonylurea drugs (acetohexamide, tolbutamide and glibenclamide) act through 'sulfonylurea receptors'; tritiated glibenclamide can be used to identify a 140 kDa protein called SUR1 (now known as ABCC8) [2].

References

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1. Aguilar-Bryan L, Clement JP, Gonzalez G, Kunjilwar K, Babenko A, Bryan J. (1998) Toward understanding the assembly and structure of KATP channels. Physiol Rev, 78 (1): 227-45. [PMID:9457174]

2. Rehmann H. (2012) Epac2: a sulfonylurea receptor?. Biochem Soc Trans, 40 (1): 6-10. [PMID:22260657]

3. Wängler B, Beck C, Shiue CY, Schneider S, Schwanstecher C, Schwanstecher M, Feilen PJ, Alavi A, Rösch F, Schirrmacher R. (2004) Synthesis and in vitro evaluation of (S)-2-([11C]methoxy)-4-[3-methyl-1-(2-piperidine-1-yl-phenyl)-butyl-carbamoyl]-benzoic acid ([11C]methoxy-repaglinide): a potential beta-cell imaging agent. Bioorg Med Chem Lett, 14 (20): 5205-9. [PMID:15380228]

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