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MGP002229

Record overview

MGPD IDMGP002229
Gene ID5162
SpeciesHomo sapiens (Human)
Gene Namepyruvate dehydrogenase (lipoamide) beta
Gene Symbol PDHB
SynonymsPDHBD; PHE1B; PDHE1-B;
Alternate namespyruvate dehydrogenase E1 component subunit beta, mitochondrial; pyruvate dehydrogenase, E1 beta polypeptide;
Chromosome3
Map Location3p21.1-p14.2
EC Number1.2.4.1
SummaryThe 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]
OrthologsView 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:
MAAVSGLVRRPLREVSGLLKRRFHWTAPAALQVTVRDAINQGMDEELERDEKVFLLGEEVAQYDGAYKVSRGLWKKYGDKRIIDTPISEMGFAGIAVGAA
MAGLRPICEFMTFNFSMQAIDQVINSAAKTYYMSGGLQPVPIVFRGPNGASAGVAAQHSQCFAAWYGHCPGLKVVSPWNSEDAKGLIKSAIRDNNPVVVL
ENELMYGVPFEFPPEAQSKDFLIPIGKAKIERQGTHITVVSHSRPVGHCLEAAAVLSKEGVECEVINMRTIRPMDMETIEASVMKTNHLVTVEGGWPQFG
VGAEICARIMEGPAFNFLDAPAVRVTGADVPMPYAKILEDNSIPQVKDIIFAIKKTLNI
 
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: 
LQVTVRDAINQGMDEELERDEKVFLLGEEVAQYDGAYKVSRGLWKKYGDKRIIDTPISEMGFAGIAVGAAMAGLRPICEFMTFNFSMQAIDQVINSAAKT
YYMSGVAAQHSQCFAAWYGHCPGLKVVSPWNSEDAKGLIKSAIRDNNPVVVLENELMYGVPFEFPPEAQSKDFLIPIGKAKIERQGTHITVVSHSRPVGH
CLEAAAVLSKEGVECEVINMRTIRPMDMETIEASVMKTNHLVTVEGGWPQFGVGAEICARIMEGPAFNFLDAPAVRVTGADVPMPYAKILEDNSIPQVKD
IIFAIKKTLNI

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: 
LQVTVRDAINQGMDEELERDEKVFLLGEEVAQYDGAYKVSRGLWKKYGDKRIIDTPISEMGFAGIAVGAAMAGLRPICEFMTFNFSMQAIDQVINSAAKT
YYMSGGLQPVPIVFRGPNGASAGVAAQHSQCFAAWYGHCPGLKVVSPWNSEDAKGLIKSAIRDNNPVVVLENELMYGVPFEFPPEAQSKDFLIPIGKAKI
ERQGTHITVVSHSRPVGHCLEAAAVLSKEGVECEVINMRTIRPMDMETIEASVMKTNHLVTVEGGWPQFGVGAEICARIMEGPAFNFLDAPAVRVTGADV
PMPYAKILEDNSIPQVKDIIFAIKKTLNI
 
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:
MAAVSGLVRRPLREVSGLLKRRFHWTAPAALQVTVRDAINQGMDEELERDEKVFLLGEEVAQYDGAYKVSRGLWKKYGDKRIIDTPISEMGFAGIAVGAA
MAGLRPICEFMTFNFSMQAIDQVINSAAKTYYMSGVAAQHSQCFAAWYGHCPGLKVVSPWNSEDAKGLIKSAIRDNNPVVVLENELMYGVPFEFPPEAQS
KDFLIPIGKAKIERQGTHITVVSHSRPVGHCLEAAAVLSKEGVECEVINMRTIRPMDMETIEASVMKTNHLVTVEGGWPQFGVGAEICARIMEGPAFNFL
DAPAVRVTGADVPMPYAKILEDNSIPQVKDIIFAIKKTLNI
 
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: 
LQVTVRDAINQGMDEELERDEKVFLLGEEVAQYDGAYKVSRGLWKKYGDKRIIDTPISEMGFAGIAVGAAMAGLRPICEFMTFNFSMQAIDQVINSAAKT
YYMSGVAAQHSQCFAAWYGHCPGLKVVSPWNSEDAKGLIKSAIRDNNPVVVLENELMYGVPFEFPPEAQSKDFLIPIGKAKIERQGTHITVVSHSRPVGH
CLEAAAVLSKEGVECEVINMRTIRPMDMETIEASVMKTNHLVTVEGGWPQFGVGAEICARIMEGPAFNFLDAPAVRVTGADVPMPYAKILEDNSIPQVKD
IIFAIKKTLNI
 
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