Primary Information |
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| BoMiProt ID | Bomi3500 |
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| Protein Name | 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial/3-hydroxyisobutyryl-coenzyme A hydrolase/HIB-CoA hydrolase/HIBYL-CoA-H |
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| Organism | Bos taurus |
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| Uniprot ID | Q2HJ73 |
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| Milk Fraction | Whey |
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| Ref Sequence ID | XP_010800133.1 |
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| Aminoacid Length | 386 |
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| Molecular Weight | 43349 |
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| FASTA Sequence |
Download |
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| Gene Name | HIBCH |
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| Protein Existence Status | reviewed |
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Secondary Information |
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| Protein Function | This protein controls the formation of 3-hydroxyisobutyrate (3-HIB) in the valine degradation pathway. |
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| PTMs | N6-Acetylation at Lys,Phosphorylation at Ser |
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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
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| Predicted Disorder Regions | NA |
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| DisProt Annotation | |
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| TM Helix Prediction | No TM helices |
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| Significance of PTMs | 19S RP ATPase subunit Rpt6 was phosphorylated, the activity of proteasome was regulated by cAMP-dependent protein kinase (PKA). |
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| 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. |
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| 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. |
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