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Reviewed, UniProtKB/Swiss-Prot P31937 (3HIDH_HUMAN)

Last modified June 16, 2009. Version 94. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (4) | Third-party data | Customize display text xml rdf/xml gff fasta
Names and origin · Protein attributes · General annotation (Comments) · Ontologies · Sequence annotation (Features) · Sequences · References · Cross-references · Entry information · Relevant documents

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Names and origin

Protein namesRecommended name:
    3-hydroxyisobutyrate dehydrogenase, mitochondrial
      Short name=HIBADH
    EC=1.1.1.31
Gene names
Name: HIBADH
OrganismHomo sapiens (Human)
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo

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Protein attributes

Sequence length336 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at protein level.

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General annotation (Comments)

Catalytic activity

3-hydroxy-2-methylpropanoate + NAD+ = 2-methyl-3-oxopropanoate + NADH.

Subunit structure

Homodimer By similarity.

Subcellular location

Mitochondrion.

Sequence similarities

Belongs to the 3-hydroxyisobutyrate dehydrogenase family.

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Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Transit peptide1 – 3636Mitochondrion Ref.3
Chain37 – 3363003-hydroxyisobutyrate dehydrogenase, mitochondrial
PRO_0000007158

Regions

Nucleotide binding40 – 6829NAD
Nucleotide binding103 – 1042NAD

Sites

Active site2091 By similarity
Binding site1081NAD
Binding site1341NAD
Binding site2841NAD

Secondary structure

1 ...................................................... 336
Helix Strand Turn

Details...

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Sequences

Sequence LengthMass (Da)Tools
P31937-1 [UniParc].

Last modified January 24, 2001. Version 2.
Checksum: DA3128774A91AF48

FASTA33635,329
        10         20         30         40         50         60 
MAASLRLLGA ASGLRYWSRR LRPAAGSFAA VCSRSVASKT PVGFIGLGNM GNPMAKNLMK 

        70         80         90        100        110        120 
HGYPLIIYDV FPDACKEFQD AGEQVVSSPA DVAEKADRII TMLPTSINAI EAYSGANGIL 

       130        140        150        160        170        180 
KKVKKGSLLI DSSTIDPAVS KELAKEVEKM GAVFMDAPVS GGVGAARSGN LTFMVGGVED 

       190        200        210        220        230        240 
EFAAAQELLG CMGSNVVYCG AVGTGQAAKI CNNMLLAISM IGTAEAMNLG IRLGLDPKLL 

       250        260        270        280        290        300 
AKILNMSSGR CWSSDTYNPV PGVMDGVPSA NNYQGGFGTT LMAKDLGLAQ DSATSTKSPI 

       310        320        330 
LLGSLAHQIY RMMCAKGYSK KDFSSVFQFL REEETF

« Hide

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References

« Hide 'large scale' references
[1]"The DNA sequence of human chromosome 7."
Hillier L.W., Fulton R.S., Fulton L.A., Graves T.A., Pepin K.H., Wagner-McPherson C., Layman D., Maas J., Jaeger S., Walker R., Wylie K., Sekhon M., Becker M.C., O'Laughlin M.D., Schaller M.E., Fewell G.A., Delehaunty K.D., Miner T.L. expand/collapse author list , Nash W.E., Cordes M., Du H., Sun H., Edwards J., Bradshaw-Cordum H., Ali J., Andrews S., Isak A., Vanbrunt A., Nguyen C., Du F., Lamar B., Courtney L., Kalicki J., Ozersky P., Bielicki L., Scott K., Holmes A., Harkins R., Harris A., Strong C.M., Hou S., Tomlinson C., Dauphin-Kohlberg S., Kozlowicz-Reilly A., Leonard S., Rohlfing T., Rock S.M., Tin-Wollam A.-M., Abbott A., Minx P., Maupin R., Strowmatt C., Latreille P., Miller N., Johnson D., Murray J., Woessner J.P., Wendl M.C., Yang S.-P., Schultz B.R., Wallis J.W., Spieth J., Bieri T.A., Nelson J.O., Berkowicz N., Wohldmann P.E., Cook L.L., Hickenbotham M.T., Eldred J., Williams D., Bedell J.A., Mardis E.R., Clifton S.W., Chissoe S.L., Marra M.A., Raymond C., Haugen E., Gillett W., Zhou Y., James R., Phelps K., Iadanoto S., Bubb K., Simms E., Levy R., Clendenning J., Kaul R., Kent W.J., Furey T.S., Baertsch R.A., Brent M.R., Keibler E., Flicek P., Bork P., Suyama M., Bailey J.A., Portnoy M.E., Torrents D., Chinwalla A.T., Gish W.R., Eddy S.R., McPherson J.D., Olson M.V., Eichler E.E., Green E.D., Waterston R.H., Wilson R.K.
Nature 424:157-164(2003) [PubMed: 12853948] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[2]"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
The MGC Project Team
Genome Res. 14:2121-2127(2004) [PubMed: 15489334] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
Tissue: Lung.
[3]"Human liver protein map: update 1993."
Hughes G.J., Frutiger S., Paquet N., Pasquali C., Sanchez J.-C., Tissot J.-D., Bairoch A., Appel R.D., Hochstrasser D.F.
Electrophoresis 14:1216-1222(1993) [PubMed: 8313870] [Abstract]
Cited for: PROTEIN SEQUENCE OF 37-47.
Tissue: Liver.
[4]Colinge J., Superti-Furga G., Bennett K.L.
Submitted (OCT-2008) to UniProtKB
Cited for: IDENTIFICATION [LARGE SCALE ANALYSIS], MASS SPECTROMETRY.
[5]"Crystal structure of human hydroxyisobutyrate dehydrogenase."
Structural genomics consortium (SGC)
Submitted (APR-2006) to the PDB data bank
Cited for: X-RAY CRYSTALLOGRAPHY (2.38 ANGSTROMS) OF 41-335 OF APOPROTEIN AND IN COMPLEX WITH NADH.

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Cross-references

Sequence databases

AC007130 Genomic DNA. No translation available.
AC005091 Genomic DNA. No translation available.
BC032324 mRNA. Translation: AAH32324.1.
IPIIPI00013860.
RefSeqNP_689953.1.
UniGeneHs.406758

3D structure databases

EntryMethodResolution (Å)ChainPositionsPDBsum
2GF2X-ray2.38A/B/C/D41-335[»]
2I9PX-ray2.55A/B/C/D41-336[»]
ModBaseSearch...

2-D gel databases

SWISS-2DPAGEP31937.
Siena-2DPAGEP31937.

Proteomic databases

PRIDEP31937.

Genome annotation databases

EnsemblENSG00000106049. Homo sapiens. [Contig view]
GeneID11112.
KEGGhsa:11112.

Organism-specific databases

GeneCardsGC07M027531.
H-InvDBHIX0006550.
HGNCHGNC:4907. HIBADH.
MIM608475. gene.
PharmGKBPA29280.
GenAtlasSearch...

Phylogenomic databases

HOGENOMP31937.
HOVERGENP31937.
OMAP31937. PMARNLI.

Enzyme and pathway databases

BRENDA1.1.1.31. 247.
ReactomeREACT_13. Metabolism of amino acids.

Gene expression databases

ArrayExpressP31937.
BgeeP31937.
CleanExHS_HIBADH.
GermOnlineENSG00000106049. Homo sapiens.

Family and domain databases

InterProIPR002204. 3-OH-isobutyrate_DH-rel_CS.
IPR015815. 3hydroxyacid_DH/Rdtase.
IPR006183. 6-phosphogluconate_DH.
IPR006115. 6PGDH_NAD-bd.
IPR013328. DH_multihelical.
IPR011548. IsoBut3OH_DH.
IPR016040. NAD(P)-bd_dom.
[Graphical view]
Gene3DG3DSA:3.40.50.720. NAD(P)-bd. 1 hit.
G3DSA:1.10.1040.10. Opine_DH. 1 hit.
PANTHERPTHR22981. 3hydroxy_acid_DH. 1 hit.
PfamPF03446. NAD_binding_2. 1 hit.
[Graphical view]
PRINTSPR00076. 6PGDHDRGNASE.
TIGRFAMsTIGR01692. HIBADH. 1 hit.
PROSITEPS00895. 3_HYDROXYISOBUT_DH. 1 hit.
[Graphical view]
ProtoNetSearch...

Other Resources

DrugBankDB00157. NADH.
NextBio42238.
SOURCESearch...

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Entry information

Entry name3HIDH_HUMAN
AccessionPrimary (citable) accession number: P31937
Secondary accession number(s): Q9UDN3
Entry history
Integrated into UniProtKB/Swiss-Prot: July 1, 1993
Last sequence update: January 24, 2001
Last modified: June 16, 2009
This is version 94 of the entry and version 2 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation projectHPI (Human Proteome Initiative)

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Relevant documents

Human chromosome 7

Human chromosome 7: entries, gene names and cross-references to MIM

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

PDB cross-references

Index of Protein Data Bank (PDB) cross-references

SIMILARITY comments

Index of protein domains and families

Names and origin · Protein attributes · General annotation (Comments) · Ontologies · Sequence annotation (Features) · Sequences · References · Cross-references · Entry information · Relevant documents