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BCKDHB branched chain keto acid dehydrogenase E1 subunit beta [ Canis lupus familiaris (dog) ]

Gene ID: 474978, updated on 17-Aug-2024

Summary

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Official Symbol
BCKDHBprovided by VGNC
Official Full Name
branched chain keto acid dehydrogenase E1 subunit betaprovided by VGNC
Primary source
VGNC:VGNC:38406
Gene type
protein coding
RefSeq status
MODEL
Organism
Canis lupus familiaris (sub-species: familiaris)
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Laurasiatheria; Carnivora; Caniformia; Canidae; Canis
Orthologs
human mouse all
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Genomic context

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Location:
chromosome: 12
Exon count:
11
Annotation release Status Assembly Chr Location
106 current ROS_Cfam_1.0 (GCF_014441545.1) 12 NC_051816.1 (41775384..41991512)
106 current Dog10K_Boxer_Tasha (GCF_000002285.5) 12 NC_006594.4 (40881519..41096408)
106 current UU_Cfam_GSD_1.0 (GCF_011100685.1) 12 NC_049233.1 (41216793..41431727)
106 current UMICH_Zoey_3.1 (GCF_005444595.1) 12 NC_049272.1 (41088764..41303619)
106 current UNSW_CanFamBas_1.0 (GCF_013276365.1) 12 NC_049753.1 (41063571..41271816)

Chromosome 12 - NC_051816.1Genomic Context describing neighboring genes Neighboring gene uncharacterized LOC106559592 Neighboring gene TTK protein kinase Neighboring gene uncharacterized LOC102155357 Neighboring gene uncharacterized LOC111098335 Neighboring gene 40S ribosomal protein S17-like

Genomic regions, transcripts, and products

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Go to nucleotide: Graphics FASTA GenBank

Bibliography

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GeneRIFs: Gene References Into Functions

General gene information

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Markers

Homology

Gene Ontology Provided by RefSeq

Function Evidence Code Pubs
enables catalytic activity IEA
Inferred from Electronic Annotation
more info
 PubMed 
Process Evidence Code Pubs
involved_in branched-chain amino acid catabolic process IEA
Inferred from Electronic Annotation
more info
 PubMed 
involved_in response to nutrient IEA
Inferred from Electronic Annotation
more info
 PubMed 

General protein information

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Preferred Names
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial; LOW QUALITY PROTEIN: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
Names
branched chain keto acid dehydrogenase E1, beta polypeptide (maple syrup urine disease)
XP_005627622.1
XP_005627623.1
XP_038348239.1
XP_038348240.1
XP_038348242.1
XP_038348243.1
XP_038410533.1
XP_038410534.1
XP_038410535.1
XP_038410536.1
XP_038474389.1
XP_038474390.1
XP_038474391.1
XP_038474392.1
XP_038474393.1
XP_038530765.1
XP_038539954.1
XP_532213.2

NCBI Reference Sequences (RefSeq)

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RefSeqs of Annotated Genomes: Canis lupus familiaris Annotation Release 106 details...Open this link in a new tab

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference ROS_Cfam_1.0

Genomic

  1. NC_051816.1 Reference ROS_Cfam_1.0

    Range
    41775384..41991512
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_038684026.1 → XP_038539954.1  LOW QUALITY PROTEIN: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X1

    UniProtKB/TrEMBL
    A0A8C0KIL5, A0A8C0MFJ7
    Conserved Domains (1) summary
    PTZ00182
    Location:61 → 383
    PTZ00182; 3-methyl-2-oxobutanate dehydrogenase; Provisional

Alternate UMICH_Zoey_3.1

Genomic

  1. NC_049272.1 Alternate UMICH_Zoey_3.1

    Range
    41088764..41303619
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_038492315.1 → XP_038348243.1  2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X4

    Conserved Domains (1) summary
    PTZ00182
    Location:61 → 333
    PTZ00182; 3-methyl-2-oxobutanate dehydrogenase; Provisional

  2. XM_038492314.1 → XP_038348242.1  2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X3

    Conserved Domains (1) summary
    PTZ00182
    Location:61 → 342
    PTZ00182; 3-methyl-2-oxobutanate dehydrogenase; Provisional

  3. XM_038492312.1 → XP_038348240.1  2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X2

  4. XM_038492311.1 → XP_038348239.1  2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X2

Alternate UNSW_CanFamBas_1.0

Genomic

  1. NC_049753.1 Alternate UNSW_CanFamBas_1.0

    Range
    41063571..41271816
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_038618465.1 → XP_038474393.1  2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X7

    Conserved Domains (1) summary
    PTZ00182
    Location:61 → 333
    PTZ00182; 3-methyl-2-oxobutanate dehydrogenase; Provisional

  2. XM_038618463.1 → XP_038474391.1  2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X6

    Conserved Domains (1) summary
    PTZ00182
    Location:61 → 342
    PTZ00182; 3-methyl-2-oxobutanate dehydrogenase; Provisional

  3. XM_038618464.1 → XP_038474392.1  2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X6

    Conserved Domains (1) summary
    PTZ00182
    Location:61 → 342
    PTZ00182; 3-methyl-2-oxobutanate dehydrogenase; Provisional

  4. XM_038618462.1 → XP_038474390.1  2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X5

  5. XM_038618461.1 → XP_038474389.1  2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X5

Alternate UU_Cfam_GSD_1.0

Genomic

  1. NC_049233.1 Alternate UU_Cfam_GSD_1.0

    Range
    41216793..41431727
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_038554608.1 → XP_038410536.1  2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X10

    Conserved Domains (1) summary
    PTZ00182
    Location:61 → 333
    PTZ00182; 3-methyl-2-oxobutanate dehydrogenase; Provisional

  2. XM_038554607.1 → XP_038410535.1  2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X9

    UniProtKB/TrEMBL
    A0A8C0KIL5, A0A8C0MFJ7
    Conserved Domains (1) summary
    PTZ00182
    Location:61 → 342
    PTZ00182; 3-methyl-2-oxobutanate dehydrogenase; Provisional

  3. XM_038554606.1 → XP_038410534.1  2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X8

    UniProtKB/TrEMBL
    A0A8C0KIL5, A0A8C0MFJ7, A0A8P0N5V9

  4. XM_038554605.1 → XP_038410533.1  2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X8

    UniProtKB/TrEMBL
    A0A8C0KIL5, A0A8C0MFJ7, A0A8P0N5V9

Alternate Dog10K_Boxer_Tasha Primary Assembly

Genomic

  1. NC_006594.4 Alternate Dog10K_Boxer_Tasha Primary Assembly

    Range
    40881519..41096408
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_038674837.1 → XP_038530765.1  2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X13

    Conserved Domains (1) summary
    PTZ00182
    Location:61 → 333
    PTZ00182; 3-methyl-2-oxobutanate dehydrogenase; Provisional

  2. XM_005627566.4 → XP_005627623.1  2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X12

    UniProtKB/TrEMBL
    A0A8C0KIL5, A0A8C0MFJ7
    Conserved Domains (1) summary
    PTZ00182
    Location:61 → 342
    PTZ00182; 3-methyl-2-oxobutanate dehydrogenase; Provisional

  3. XM_005627565.4 → XP_005627622.1  2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X11

    See identical proteins and their annotated locations for XP_005627622.1

    UniProtKB/TrEMBL
    A0A8C0KIL5, A0A8C0MFJ7, A0A8P0N5V9
    Conserved Domains (1) summary
    PTZ00182
    Location:61 → 385
    PTZ00182; 3-methyl-2-oxobutanate dehydrogenase; Provisional

  4. XM_532213.7 → XP_532213.2  2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X11

    See identical proteins and their annotated locations for XP_532213.2

    UniProtKB/TrEMBL
    A0A8C0KIL5, A0A8C0MFJ7, A0A8P0N5V9
    Conserved Domains (1) summary
    PTZ00182
    Location:61 → 385
    PTZ00182; 3-methyl-2-oxobutanate dehydrogenase; Provisional
Nucleotide Protein
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