Reactome | UniProt:O43837 IDH3B

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UniProt:O43837 IDH3B Show undefined attributes

chain	transit peptide:1-34 chain:35-385
checksum	7324E6CC30A68EE2
comment	FUNCTION Plays a structural role to facilitate the assembly and ensure the full activity of the enzyme catalyzing the decarboxylation of isocitrate (ICT) into alpha-ketoglutarate. The heterodimer composed of the alpha (IDH3A) and beta (IDH3B) subunits and the heterodimer composed of the alpha (IDH3A) and gamma (IDH3G) subunits, have considerable basal activity but the full activity of the heterotetramer (containing two subunits of IDH3A, one of IDH3B and one of IDH3G) requires the assembly and cooperative function of both heterodimers.ACTIVITY REGULATION The heterotetramer and the heterodimer composed of IDH3A and IDH3G subunits can be allosterically activated by citrate (CIT) or/and ADP, and the two activators can act independently or synergistically. The heterodimer composed of IDH3A and IDH3B subunits cannot be allosterically regulated and the allosteric regulation of the heterotetramer is through the IDH3G subunit and not the IDH3B subunit. The IDH3G subunit contains the allosteric site which consists of a CIT-binding site and an ADP-binding site, and the binding of CIT and ADP causes conformational changes at the allosteric site which are transmitted to the active site in the catalytic subunit (IDH3A) through a cascade of conformational changes at the heterodimer interface, leading to stabilization of the isocitrate-binding at the active site and thus activation of the enzyme. ATP can activate the heterotetramer and the heterodimer composed of IDH3A and IDH3G subunits at low concentrations but inhibits their activities at high concentrations, whereas ATP exhibits only inhibitory effect on the heterodimer composed of IDH3A and IDH3B subunits.SUBUNIT Heterooligomer of subunits alpha (IDH3A), beta (IDH3B), and gamma (IDH3G) in the apparent ratio of 2:1:1. The heterodimer containing one IDH3A and one IDH3B subunit and the heterodimer containing one IDH3A and one IDH3G subunit assemble into a heterotetramer (which contains two subunits of IDH3A, one of IDH3B and one of IDH3G) and further into the heterooctamer.INTERACTION The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the isocitrate and isopropylmalate dehydrogenases family.
crossReference	[DatabaseIdentifier:12413533] RefSeq:NP_008830.2 [DatabaseIdentifier:12413532] RefSeq:NP_777280.1 [DatabaseIdentifier:12413531] RefSeq:NP_001317692.1 [DatabaseIdentifier:12285390] Pharos - Targets:O43837 [DatabaseIdentifier:12245036] PRO:O43837 [DatabaseIdentifier:12172364] PDB:6KDY [DatabaseIdentifier:12172368] PDB:8GRD [DatabaseIdentifier:12172354] PDB:7CE3 [DatabaseIdentifier:12172361] PDB:6KDE [DatabaseIdentifier:12172359] PDB:8GS5 [DatabaseIdentifier:12172358] PDB:8GRU [DatabaseIdentifier:12172366] PDB:6KE3 [DatabaseIdentifier:12172357] PDB:8GRB [DatabaseIdentifier:12172360] PDB:6KDF [DatabaseIdentifier:12136400] Orphanet:IDH3B [DatabaseIdentifier:12125082] OpenTargets:ENSG00000101365 [DatabaseIdentifier:11840542] HPA:ENSG00000101365-IDH3B [DatabaseIdentifier:11831597] HMDB Protein:HMDBP00897 [DatabaseIdentifier:11802908] GlyGen:O43837 [DatabaseIdentifier:11787471] GeneCards:IDH3B [DatabaseIdentifier:11722575] ENSEMBL:ENST00000380843 [DatabaseIdentifier:11722574] ENSEMBL:ENST00000380851 [DatabaseIdentifier:11599876] ENSEMBL:ENSP00000370223 [DatabaseIdentifier:11599875] ENSEMBL:ENSP00000370232 [DatabaseIdentifier:11492629] ENSEMBL:ENSG00000101365
databaseName	UniProt
dbId	57064
description	recommendedName: Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial alternativeName: Isocitric dehydrogenase subunit beta alternativeName: NAD(+)-specific ICDH subunit beta
displayName	UniProt:O43837 IDH3B
geneName	IDH3B
identifier	O43837
isSequenceChanged	false
keyword	3D-structure Acetylation Alternative splicing Disease variant Mitochondrion Proteomics identification Reference proteome Retinitis pigmentosa Transit peptide Tricarboxylic acid cycle
modified	[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
moleculeType	Protein
name	IDH3B
otherIdentifier	11730186_a_at 11730187_x_at 11733576_a_at 11744207_a_at 11745811_a_at 11745812_x_at 16916574 201509_PM_at 201509_at 210014_PM_x_at 210014_x_at 210418_PM_s_at 210418_s_at 3420 3895076 3895077 3895078 3895079 3895080 3895081 3895082 3895083 3895084 3895085 3895086 3895087 3895088 3895089 3895090 3895091 3895092 3895093 3895094 3895095 3895096 3895097 3895099 3895100 3895102 3895103 3895106 3895107 40110_at 40111_g_at 40112_at 8064522 A_19_P00809916 A_23_P166068 A_23_P502196 A_33_P3261084 A_33_P3358069 GE81693 GO:0000287 GO:0004449 GO:0005515 GO:0005634 GO:0005739 GO:0005759 GO:0006099 GO:0006102 GO:0009055 GO:0016491 GO:0016616 GO:0045242 GO:0051287 HMNXSV003013485 HMNXSV003037279 ILMN_1721669 ILMN_1810400 ILMN_2373632 PH_hs_0020528 PH_hs_0035995 TC20000563.hg TC20001384.hg g4103445_3p_a_at
physicalEntity	[EntityWithAccessionedSequence:R-HSA-70963] IDH3B [mitochondrial matrix] - Homo sapiens
referenceDatabase	[ReferenceDatabase:2] UniProt
referenceGene	[ReferenceDNASequence:12751250] UCSC:O43837 IDH3B [ReferenceDNASequence:12110430] OMIM:604526 IDH3B [ReferenceDNASequence:12072045] NCBI Gene:3420 IDH3B [ReferenceDNASequence:11987952] Monarch:3420 IDH3B [ReferenceDNASequence:11897445] KEGG:hsa:3420 IDH3B [ReferenceDNASequence:11819111] HGNC:5385 IDH3B [ReferenceDNASequence:11439031] dbSNP Gene:3420 IDH3B [ReferenceDNASequence:11413200] CTD Gene:3420 IDH3B [ReferenceDNASequence:11401665] COSMIC - genes:IDH3B IDH3B [ReferenceDNASequence:11358163] BioGPS Gene:3420 IDH3B [ReferenceDNASequence:ensembl:ENSG00000101365] ENSEMBL:ENSG00000101365 IDH3B
referenceTranscript	[ReferenceRNASequence:12595230] RefSeq:NM_001330763.1 IDH3B [ReferenceRNASequence:12595232] RefSeq:NM_006899.4 IDH3B [ReferenceRNASequence:12595231] RefSeq:NM_174855.4 IDH3B
schemaClass	ReferenceGeneProduct
secondaryIdentifier	IDH3B_HUMAN B2RDR1 D3DVX2 D3DVX3 O95106 Q5JXS8 Q9NQ06 Q9NQ07 Q9NUZ0 Q9UEX0 Q9UG99
sequenceLength	385
species	[Species:48887] Homo sapiens
stId	uniprot:O43837
url	http://purl.uniprot.org/uniprot/O43837

Referrals

(interactor)	[UndirectedInteraction:11208269] UniProt:P01106 <-> UniProt:O43837 (IntAct) [UndirectedInteraction:11149295] UniProt:P13569 <-> UniProt:O43837 (IntAct)
(referenceEntity)	[EntityWithAccessionedSequence:R-HSA-70963] IDH3B [mitochondrial matrix]