Gene/Proteome Database (LMPD)

LMPD ID
LMP005062
Gene ID
5162
Species
Homo sapiens (Human)
Gene Name
pyruvate dehydrogenase (lipoamide) beta
Gene Symbol
PDHB
Synonyms
PDHBD; PDHE1-B; PHE1B
Alternate Names
pyruvate dehydrogenase E1 component subunit beta, mitochondrial; pyruvate dehydrogenase, E1 beta polypeptide
Chromosome
3
Map Location
3p21.1-p14.2
EC Number
1.2.4.1
Summary
The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the overall conversion of pyruvate to acetyl-CoA and carbon dioxide, and provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle. The PDH complex is composed of multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). The E1 enzyme is a heterotetramer of two alpha and two beta subunits. This gene encodes the E1 beta subunit. Mutations in this gene are associated with pyruvate dehydrogenase E1-beta deficiency. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Mar 2012]
Orthologs
View orthologs and multiple alignments for PDHB

Proteins

pyruvate dehydrogenase E1 component subunit beta, mitochondrial isoform 1 precursor
Refseq ID NP_000916
Protein GI 156564403
UniProt ID P11177
mRNA ID NM_000925
Length 359
RefSeq Status REVIEWED
MAAVSGLVRRPLREVSGLLKRRFHWTAPAALQVTVRDAINQGMDEELERDEKVFLLGEEVAQYDGAYKVSRGLWKKYGDKRIIDTPISEMGFAGIAVGAAMAGLRPICEFMTFNFSMQAIDQVINSAAKTYYMSGGLQPVPIVFRGPNGASAGVAAQHSQCFAAWYGHCPGLKVVSPWNSEDAKGLIKSAIRDNNPVVVLENELMYGVPFEFPPEAQSKDFLIPIGKAKIERQGTHITVVSHSRPVGHCLEAAAVLSKEGVECEVINMRTIRPMDMETIEASVMKTNHLVTVEGGWPQFGVGAEICARIMEGPAFNFLDAPAVRVTGADVPMPYAKILEDNSIPQVKDIIFAIKKTLNI
pyruvate dehydrogenase E1 component subunit beta, mitochondrial isoform 2 precursor
Refseq ID NP_001166939
Protein GI 291084858
UniProt ID P11177
mRNA ID NM_001173468
Length 341
RefSeq Status REVIEWED
MAAVSGLVRRPLREVSGLLKRRFHWTAPAALQVTVRDAINQGMDEELERDEKVFLLGEEVAQYDGAYKVSRGLWKKYGDKRIIDTPISEMGFAGIAVGAAMAGLRPICEFMTFNFSMQAIDQVINSAAKTYYMSGVAAQHSQCFAAWYGHCPGLKVVSPWNSEDAKGLIKSAIRDNNPVVVLENELMYGVPFEFPPEAQSKDFLIPIGKAKIERQGTHITVVSHSRPVGHCLEAAAVLSKEGVECEVINMRTIRPMDMETIEASVMKTNHLVTVEGGWPQFGVGAEICARIMEGPAFNFLDAPAVRVTGADVPMPYAKILEDNSIPQVKDIIFAIKKTLNI
transit_peptide: 1..30 calculated_mol_wt: 3347 peptide sequence: MAAVSGLVRRPLREVSGLLKRRFHWTAPAA mat_peptide: 31..359 product: pyruvate dehydrogenase E1 component subunit beta, mitochondrial isoform 1 calculated_mol_wt: 35905 peptide sequence: LQVTVRDAINQGMDEELERDEKVFLLGEEVAQYDGAYKVSRGLWKKYGDKRIIDTPISEMGFAGIAVGAAMAGLRPICEFMTFNFSMQAIDQVINSAAKTYYMSGGLQPVPIVFRGPNGASAGVAAQHSQCFAAWYGHCPGLKVVSPWNSEDAKGLIKSAIRDNNPVVVLENELMYGVPFEFPPEAQSKDFLIPIGKAKIERQGTHITVVSHSRPVGHCLEAAAVLSKEGVECEVINMRTIRPMDMETIEASVMKTNHLVTVEGGWPQFGVGAEICARIMEGPAFNFLDAPAVRVTGADVPMPYAKILEDNSIPQVKDIIFAIKKTLNI transit_peptide: 1..30 calculated_mol_wt: 3347 peptide sequence: MAAVSGLVRRPLREVSGLLKRRFHWTAPAA mat_peptide: 31..341 product: pyruvate dehydrogenase E1 component subunit beta, mitochondrial isoform 2 calculated_mol_wt: 34186 peptide sequence: LQVTVRDAINQGMDEELERDEKVFLLGEEVAQYDGAYKVSRGLWKKYGDKRIIDTPISEMGFAGIAVGAAMAGLRPICEFMTFNFSMQAIDQVINSAAKTYYMSGVAAQHSQCFAAWYGHCPGLKVVSPWNSEDAKGLIKSAIRDNNPVVVLENELMYGVPFEFPPEAQSKDFLIPIGKAKIERQGTHITVVSHSRPVGHCLEAAAVLSKEGVECEVINMRTIRPMDMETIEASVMKTNHLVTVEGGWPQFGVGAEICARIMEGPAFNFLDAPAVRVTGADVPMPYAKILEDNSIPQVKDIIFAIKKTLNI

Gene Information

Entrez Gene ID
5162
Gene Name
pyruvate dehydrogenase (lipoamide) beta
Gene Symbol
PDHB
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0070062 IDA:UniProt C extracellular vesicular exosome
GO:0005759 TAS:Reactome C mitochondrial matrix
GO:0005634 IDA:UniProt C nucleus
GO:0045254 IDA:UniProtKB C pyruvate dehydrogenase complex
GO:0004739 TAS:ProtInc F pyruvate dehydrogenase (acetyl-transferring) activity
GO:0004738 IDA:UniProtKB F pyruvate dehydrogenase activity
GO:0006086 IDA:UniProtKB P acetyl-CoA biosynthetic process from pyruvate
GO:0044237 TAS:Reactome P cellular metabolic process
GO:0006006 IEA:UniProtKB-KW P glucose metabolic process
GO:0006090 TAS:Reactome P pyruvate metabolic process
GO:0010510 TAS:Reactome P regulation of acetyl-CoA biosynthetic process from pyruvate
GO:0044281 TAS:Reactome P small molecule metabolic process
GO:0006099 IDA:UniProtKB P tricarboxylic acid cycle

KEGG Pathway Links

KEGG Pathway ID Description
hsa01200 Carbon metabolism
hsa00020 Citrate cycle (TCA cycle)
hsa00010 Glycolysis / Gluconeogenesis
hsa04066 HIF-1 signaling pathway

Domain Information

InterPro Annotations

Accession Description
IPR027110 Pyruvate dehydrogenase E1 component subunit beta
IPR029061 Thiamin diphosphate-binding fold
IPR005476 Transketolase, C-terminal
IPR009014 Transketolase, C-terminal/Pyruvate-ferredoxin oxidoreductase, domain II
IPR005475 Transketolase-like, pyrimidine-binding domain

UniProt Annotations

Entry Information

Gene Name
pyruvate dehydrogenase (lipoamide) beta
Protein Entry
ODPB_HUMAN
UniProt ID
P11177
Species
Human

Comments

Comment Type Description
Alternative Products Event=Alternative splicing; Named isoforms=3; Name=1; IsoId=P11177-1; Sequence=Displayed; Name=2; IsoId=P11177-2; Sequence=VSP_012675; Note=No experimental confirmation available.; Name=3; IsoId=P11177-3; Sequence=VSP_043364; Note=No experimental confirmation available.;
Catalytic Activity Pyruvate + [dihydrolipoyllysine-residue acetyltransferase] lipoyllysine = [dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + CO(2).
Cofactor Name=thiamine diphosphate; Xref=ChEBI
Disease Pyruvate dehydrogenase E1-beta deficiency (PDHBD) [MIM
Function The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle.
Interaction P10515:DLAT; NbExp=7; IntAct=EBI-1035872, EBI-2959723;
Subcellular Location Mitochondrion matrix.
Subunit Heterotetramer of two PDHA1 and two PDHB subunits. The heterotetramer interacts with DLAT, and is part of the multimeric pyruvate dehydrogenase complex that contains multiple copies of pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (DLAT, E2) and lipoamide dehydrogenase (DLD, E3). These subunits are bound to an inner core composed of about 48 DLAT and 12 PDHX molecules. {ECO

Identical and Related Proteins

Unique RefSeq proteins for LMP005062 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
156564403 RefSeq NP_000916 359 pyruvate dehydrogenase E1 component subunit beta, mitochondrial isoform 1 precursor
291084858 RefSeq NP_001166939 341 pyruvate dehydrogenase E1 component subunit beta, mitochondrial isoform 2 precursor

Identical Sequences to LMP005062 proteins

Reference Database Accession Length Protein Name
GI:291084858 DBBJ BAG56698.1 341 unnamed protein product [Homo sapiens]
GI:156564403 GenBank AGO72391.1 359 Sequence 69 from patent US 8470972
GI:156564403 GenBank AGU32382.1 359 Sequence 69 from patent US 8507277
GI:156564403 GenBank AGX48286.1 359 Sequence 69 from patent US 8541550
GI:156564403 GenBank AIC49357.1 359 PDHB, partial [synthetic construct]
GI:156564403 RefSeq XP_005547548.1 359 PREDICTED: pyruvate dehydrogenase E1 component subunit beta, mitochondrial isoform X1 [Macaca fascicularis]
GI:291084858 RefSeq XP_005547549.1 341 PREDICTED: pyruvate dehydrogenase E1 component subunit beta, mitochondrial isoform X2 [Macaca fascicularis]
GI:156564403 RefSeq XP_007982978.1 359 PREDICTED: pyruvate dehydrogenase E1 component subunit beta, mitochondrial [Chlorocebus sabaeus]

Related Sequences to LMP005062 proteins

Reference Database Accession Length Protein Name
GI:156564403 GenBank AAA60053.1 359 pyruvate dehydrogenase E1-beta subunit [Homo sapiens]
GI:156564403 GenBank AAA60233.1 359 pyruvate dehydrogenase E1-beta subunit precursor [Homo sapiens]
GI:291084858 GenBank AAH00439.1 359 Pyruvate dehydrogenase (lipoamide) beta [Homo sapiens]
GI:291084858 GenBank AAH01924.1 359 Pyruvate dehydrogenase (lipoamide) beta [Homo sapiens]
GI:156564403 GenBank AAV20644.1 359 Sequence 904 from patent US 6753314
GI:156564403 GenBank AAX37011.1 360 pyruvate dehydrogenase beta, partial [synthetic construct]
GI:156564403 GenBank EHH25549.1 359 hypothetical protein EGK_21392 [Macaca mulatta]
GI:291084858 GenBank JAA00740.1 359 pyruvate dehydrogenase (lipoamide) beta [Pan troglodytes]
GI:291084858 GenBank JAA14504.1 359 pyruvate dehydrogenase (lipoamide) beta [Pan troglodytes]
GI:291084858 GenBank JAA39298.1 359 pyruvate dehydrogenase (lipoamide) beta [Pan troglodytes]
GI:156564403 RefSeq NP_001182323.1 359 pyruvate dehydrogenase E1 component subunit beta, mitochondrial [Macaca mulatta]
GI:291084858 SwissProt P11177.3 359 RecName: Full=Pyruvate dehydrogenase E1 component subunit beta, mitochondrial; Short=PDHE1-B; Flags: Precursor [Homo sapiens]