Gene and Protein Information
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 member 8
Rat	17	1582	1q22	Abcc8	ATP binding cassette subfamily C member 8

Previous and Unofficial Names

ABC36 | ATP-binding cassette | ATP-binding cassette, subfamily C (CFTR/MRP), member 8 | HHF1 | HI | MRP8 | PHHI | SUR1 | TNDM2

Database Links
Alphafold	Q09428 (Hs), Q09429 (Rn)
ChEMBL Target	CHEMBL2071 (Hs), CHEMBL1944490 (Rn)
DrugBank Target	Q09428 (Hs)
Ensembl Gene	ENSG00000006071 (Hs), ENSMUSG00000040136 (Mm), ENSRNOG00000021130 (Rn)
Entrez Gene	6833 (Hs), 20927 (Mm), 25559 (Rn)
Human Protein Atlas	ENSG00000006071 (Hs)
KEGG Gene	hsa:6833 (Hs), mmu:20927 (Mm), rno:25559 (Rn)
OMIM	600509 (Hs)
Orphanet	ORPHA117665 (Hs)
Pharos	Q09428 (Hs)
RefSeq Nucleotide	NM_000352 (Hs), NM_011510 (Mm), NM_013039 (Rn)
RefSeq Protein	NP_000343 (Hs), NP_035640 (Mm), NP_037171 (Rn)
UniProtKB	Q09428 (Hs), Q09429 (Rn)
Wikipedia	ABCC8 (Hs)

Download all structure-activity data for this target as a CSV file

Inhibitors
Key to terms and symbols	View all chemical structures	Click column headers to sort
Ligand Sp. Action Value Parameter Reference repaglinide Hs Inhibition 7.0 pIC50 3 ⤷ pIC50 7.0 (IC50 1.06x10-7 M) [3] chlorpropamide 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

Clinically-Relevant Mutations and Pathophysiology
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:  Glucagon
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].