Recombinant Human 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial (HIBCH)

Code CSB-YP747360HU
MSDS
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Source Yeast
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Code CSB-EP747360HU
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Source E.coli
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Code CSB-EP747360HU-B
MSDS
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Source E.coli
Conjugate Avi-tag Biotinylated
E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.
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Code CSB-BP747360HU
MSDS
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Source Baculovirus
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Code CSB-MP747360HU
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Source Mammalian cell
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Product Details

Purity
>85% (SDS-PAGE)
Target Names
HIBCH
Uniprot No.
Alternative Names
3 hydroxyisobutyryl Coenzyme A hydrolase ; 3 hydroxyisobutyryl Coenzyme A hydrolase; mitochondrial ; 3-hydroxyisobutyryl-CoA hydrolase; 3-hydroxyisobutyryl-coenzyme A hydrolase; BETA HYDROXYISOBUTYRYL COENZYME A HYDROLASE; HIB CoA hydrolase; HIB-CoA hydrolase; HIBCH; HIBCH_HUMAN; HIBCoA hydrolase; HIBYL CoA H; HIBYL CoAH; HIBYL-CoA-H; HIBYLCoA H; HIBYLCoAH; mitochondrial
Species
Homo sapiens (Human)
Expression Region
33-386
Target Protein Sequence
DAAEEVLL EKKGCTGVIT LNRPKFLNAL TLNMIRQIYP QLKKWEQDPE TFLIIIKGAG GKAFCAGGDI RVISEAEKAK QKIAPVFFRE EYMLNNAVGS CQKPYVALIH GITMGGGVGL SVHGQFRVAT EKCLFAMPET AIGLFPDVGG GYFLPRLQGK LGYFLALTGF RLKGRDVYRA GIATHFVDSE KLAMLEEDLL ALKSPSKENI ASVLENYHTE SKIDRDKSFI LEEHMDKINS CFSANTVEEI IENLQQDGSS FALEQLKVIN KMSPTSLKIT LRQLMEGSSK TLQEVLTMEY RLSQACMRGH DFHEGVRAVL IDKDQSPKWK PADLKEVTEE DLNNHFKSLG SSDLKF
Protein Length
Full Length of Mature Protein
Tag Info
Tag type will be determined during the manufacturing process.
The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
Form
Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
Buffer before Lyophilization
Tris/PBS-based buffer, 6% Trehalose, pH 8.0
Reconstitution
We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
Troubleshooting and FAQs
Storage Condition
Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Shelf Life
The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
Lead Time
Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
Notes
Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet
Please contact us to get it.

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Target Background

Function
Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA.
Gene References into Functions
  1. Novel (founder) mutation in HIBCH that causes a mild phenotype, allowing survival into adulthood. PMID: 27400804
  2. Polymorphism in HIBCH is associated with HIBCH deficiency. PMID: 27132595
  3. findings demonstrated a novel homozygous pathogenic missense mutation c.950G PMID: 24299452
  4. Molecular analysis in both patients uncovered mutations in the HIBCH gene, including one missense mutation in a conserved part of the protein and two mutations affecting splicing. PMID: 17160907
Involvement in disease
3-hydroxyisobutryl-CoA hydrolase deficiency (HIBCHD)
Subcellular Location
Mitochondrion.
Protein Families
Enoyl-CoA hydratase/isomerase family
Tissue Specificity
Highly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung.
Database Links

HGNC: 4908

OMIM: 250620

KEGG: hsa:26275

STRING: 9606.ENSP00000352706

UniGene: Hs.656685

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