Search by BoMiProt ID - Bomi3500


Primary Information

BoMiProt ID Bomi3500
Protein Name 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial/3-hydroxyisobutyryl-coenzyme A hydrolase/HIB-CoA hydrolase/HIBYL-CoA-H
Organism Bos taurus
Uniprot IdQ2HJ73
Milk FractionWhey
Ref Sequence Id XP_010800133.1
Aminoacid Length 386
Molecular Weight 43349
Fasta Sequence https://www.uniprot.org/uniprot/Q2HJ73.fasta
Gene Name HIBCH
Protein Existence Status reviewed

Secondary Information

Protein Function This protein controls the formation of 3-hydroxyisobutyrate (3-HIB) in the valine degradation pathway. 
PTMs N6-Acetylation at Lys,Phosphorylation at Ser
Site(s) of PTM(s)

N-glycosylation, O-glycosylation,
Phosphorylation
>sp|Q2HJ73|HIBCH_BOVIN 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial OS=Bos taurus OX=9913 GN=HIBCH PE=2 SV=1 MGLQGLCRLMSRFNSYKRTNIILQHLKMSNHTDAAAEVLLERKGCAGVITLNRPRFLNTL TLGMIRQIYAQLKKWEQDPKTFLIIIKGAGEKAFCAGGDIRALSEARNTNQKMLQDLFRE EYILNNAIDSCQKPYIALIHGITMGGGVGVSVHGQFRVATEKSVFAMPETAIGLFPDVGG GYFLPRLQGKLGYFLALTGFRLKGRDVYTAGIATHFVDFEKLGMLEEDLLALKS*234PSKENI ADVLETYHAKSKTDQDKPFILEEHMDKINSWFSANTVEQIVDNLQQDGSSFALEQLKVIK KMSPTSLKITLRQLMEGSSKTLPEVLIMEYRLSQACMKGHDFHEGVRAVLIDKDQS*356PKWK PADLKEVTDEDLNDYFKSLGSNDLKF
Predicted Disorder Regions NA
DisProt Annotation
TM Helix Prediction No TM helices
Significance of PTMs 19S RP ATPase subunit Rpt6 was phosphorylated, the activity of proteasome was regulated by cAMP-dependent protein kinase (PKA).
Additional Comments 3-Hydroxyisobutyryl-CoA hydrolase (HIBCH) deficiency is a rare mitochondrial disorder of valine metabolism which may present with motor delay, hypotonia, ataxia, dystonia, seizures poor feeding, and organic aciduria.  HIBCH as a critical enzyme of valine catabolism in CRC progression and resistance to anti-VEGF therapy. A novel HIBCH inhibitor SBF-1, which highlights the combined therapy using valine catabolic inhibitor along with anti-VEGF drugs, to control progression of CRC.
Bibliography 1.Casano KR, Ryan ME, Bicknese AR, Mithal DS. MRI of 3-hydroxyisobutyryl-CoA hydrolase (HIBCH) deficiency. Radiol Case Rep. 2021 Jan 27;16(4):807-810. doi: 10.1016/j.radcr.2021.01.021. PMID: 33552330; PMCID: PMC7846898. 2.Shan Y, Gao Y, Jin W, Fan M, Wang Y, Gu Y, Shan C, Sun L, Li X, Yu B, Luo Q, Xu Q. Targeting HIBCH to reprogram valine metabolism for the treatment of colorectal cancer. Cell Death Dis. 2019 Aug 13;10(8):618. doi: 10.1038/s41419-019-1832-6. PMID: 31409769; PMCID: PMC6692300.