ID 3HIDH_RAT Reviewed; 335 AA. AC P29266; A1L107; DT 01-DEC-1992, integrated into UniProtKB/Swiss-Prot. DT 24-JAN-2001, sequence version 3. DT 25-NOV-2008, entry version 79. DE RecName: Full=3-hydroxyisobutyrate dehydrogenase, mitochondrial; DE Short=HIBADH; DE EC=1.1.1.31; DE Flags: Precursor; GN Name=Hibadh; OS Rattus norvegicus (Rat). OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; OC Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Sciurognathi; OC Muroidea; Muridae; Murinae; Rattus. OX NCBI_TaxID=10116; RN [1] RP NUCLEOTIDE SEQUENCE [MRNA]. RC STRAIN=Sprague-Dawley; TISSUE=Liver; RX MEDLINE=89174651; PubMed=2647728; RA Rougraff P.M., Zhang B., Kuntz M.J., Harris R.A., Crabb D.W.; RT "Cloning and sequence analysis of a cDNA for 3-hydroxyisobutyrate RT dehydrogenase. Evidence for its evolutionary relationship to other RT pyridine nucleotide-dependent dehydrogenases."; RL J. Biol. Chem. 264:5899-5903(1989). RN [2] RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. RC TISSUE=Ovary; RX PubMed=15489334; DOI=10.1101/gr.2596504; RG The MGC Project Team; RT "The status, quality, and expansion of the NIH full-length cDNA RT project: the Mammalian Gene Collection (MGC)."; RL Genome Res. 14:2121-2127(2004). RN [3] RP PROTEIN SEQUENCE OF 60-75; 297-310 AND 321-330, AND MASS SPECTROMETRY. RC STRAIN=Sprague-Dawley; TISSUE=Spinal cord; RA Lubec G., Afjehi-Sadat L.; RL Submitted (NOV-2006) to UniProtKB. RN [4] RP MUTAGENESIS. RX MEDLINE=96335606; PubMed=8766712; DOI=10.1016/0014-5793(96)00597-2; RA Hawes J.W., Harper E.T., Crabb D.W., Harris R.A.; RT "Structural and mechanistic similarities of 6-phosphogluconate and 3- RT hydroxyisobutyrate dehydrogenases reveal a new enzyme family, the 3- RT hydroxyacid dehydrogenases."; RL FEBS Lett. 389:263-267(1996). CC -!- CATALYTIC ACTIVITY: 3-hydroxy-2-methylpropanoate + NAD(+) = 2- CC methyl-3-oxopropanoate + NADH. CC -!- SUBUNIT: Homodimer. CC -!- SUBCELLULAR LOCATION: Mitochondrion. CC -!- TISSUE SPECIFICITY: Higher level in kidney, liver, and heart than CC in muscle. CC -!- SIMILARITY: Belongs to the 3-hydroxyisobutyrate dehydrogenase CC family. CC -!- SEQUENCE CAUTION: CC Sequence=AAA50312.1; Type=Frameshift; Positions=10; CC ----------------------------------------------------------------------- CC Copyrighted by the UniProt Consortium, see http://www.uniprot.org/terms CC Distributed under the Creative Commons Attribution-NoDerivs License CC ----------------------------------------------------------------------- DR EMBL; J04628; AAA50312.1; ALT_FRAME; mRNA. DR EMBL; BC127442; AAI27443.1; -; mRNA. DR PIR; A32867; A32867. DR RefSeq; NP_071579.1; -. DR UniGene; Rn.73; -. DR SMR; P29266; 40-334. DR Ensembl; ENSRNOG00000008063; Rattus norvegicus. DR GeneID; 63938; -. DR KEGG; rno:63938; -. DR RGD; 708399; Hibadh. DR HOVERGEN; P29266; -. DR NextBio; 612544; -. DR ArrayExpress; P29266; -. DR GermOnline; ENSRNOG00000008063; Rattus norvegicus. DR GO; GO:0005739; C:mitochondrion; IEA:UniProtKB-KW. DR GO; GO:0008442; F:3-hydroxyisobutyrate dehydrogenase activity; IEA:InterPro. DR GO; GO:0051287; F:NAD binding; IEA:InterPro. DR GO; GO:0004616; F:phosphogluconate dehydrogenase (decarboxyla...; IEA:InterPro. DR GO; GO:0055114; P:oxidation reduction; IEA:UniProtKB-KW. DR GO; GO:0006098; P:pentose-phosphate shunt; IEA:InterPro. DR GO; GO:0006573; P:valine metabolic process; IEA:InterPro. DR InterPro; IPR002204; 3-OH-isobutyrate_DHase-rel_CS. DR InterPro; IPR015815; 3hydroxyacid_DHase/Rdtase. DR InterPro; IPR006183; 6-phosphogluconate_DHase. DR InterPro; IPR006115; 6PGDH_NAD-bd. DR InterPro; IPR013328; DHase_multihelical. DR InterPro; IPR011548; IsoBut3OH_DH. DR InterPro; IPR016040; NAD(P)-bd. DR Gene3D; G3DSA:3.40.50.720; NAD(P)-bd; 1. DR Gene3D; G3DSA:1.10.1040.10; Opine_DH; 1. DR PANTHER; PTHR22981; 3hydroxy_acid_DH; 1. DR Pfam; PF03446; NAD_binding_2; 1. DR PRINTS; PR00076; 6PGDHDRGNASE. DR TIGRFAMs; TIGR01692; HIBADH; 1. DR PROSITE; PS00895; 3_HYDROXYISOBUT_DH; 1. PE 1: Evidence at protein level; KW Direct protein sequencing; Mitochondrion; NAD; Oxidoreductase; KW Transit peptide. FT TRANSIT 1 35 Mitochondrion (By similarity). FT CHAIN 36 335 3-hydroxyisobutyrate dehydrogenase, FT mitochondrial. FT /FTId=PRO_0000007160. FT NP_BIND 39 68 NAD (By similarity). FT NP_BIND 102 103 NAD (By similarity). FT ACT_SITE 208 208 Probable. FT BINDING 107 107 NAD (By similarity). FT BINDING 133 133 NAD; via amide nitrogen (By similarity). FT BINDING 283 283 NAD (By similarity). FT MUTAGEN 68 68 D->R: Decrease of activity with NAD, FT increase of activity with NADP. FT MUTAGEN 208 208 K->A,H,N,R: Complete loss of activity. FT MUTAGEN 212 212 N->Q: Decrease in activity. SQ SEQUENCE 335 AA; 35303 MW; D266A7838500295A CRC64; MAASLGFRGA ASGLRYWSGR RRPVGSLAAV CSRSMASKTP VGFIGLGNMG NPMAKNLIKH GYPLILYDVF PDVCKEFKEA GEQVASSPAD VAEKADRIIT MLPSSMNSIE VYSGANGILK KVKKGSLLID SSTIDPSVSK ELAKEVEKMG AVFMDAPVSG GVGAARSGNL TFMVGGVENE FAAAQELLGC MGSNVLYCGA VGSGQSAKIC NNMLLAISMI GTAEAMNLGI RSGLDPKLLA KILNMSSGRC WSSDTYNPVP GVMDGVPSSN NYQGGFGTTL MAKDLGLAQD SATSTKTPIL LGSVAHQIYR MMCSKGYSKK DFSSVFQYLR EEETF //