HIBCH Antibody

Code CSB-PA010347GA01HU
Size $600
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Product Details

Uniprot No.
Target Names
HIBCH
Alternative Names
3 hydroxyisobutyryl Coenzyme A hydrolase antibody; 3 hydroxyisobutyryl Coenzyme A hydrolase; mitochondrial antibody; 3-hydroxyisobutyryl-CoA hydrolase antibody; 3-hydroxyisobutyryl-coenzyme A hydrolase antibody; BETA HYDROXYISOBUTYRYL COENZYME A HYDROLASE antibody; HIB CoA hydrolase antibody; HIB-CoA hydrolase antibody; HIBCH antibody; HIBCH_HUMAN antibody; HIBCoA hydrolase antibody; HIBYL CoA H antibody; HIBYL CoAH antibody; HIBYL-CoA-H antibody; HIBYLCoA H antibody; HIBYLCoAH antibody; mitochondrial antibody
Raised in
Rabbit
Species Reactivity
Human,Mouse,Rat
Immunogen
Human HIBCH
Immunogen Species
Homo sapiens (Human)
Isotype
IgG
Purification Method
Antigen Affinity purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
Tested Applications
ELISA,WB,IHC
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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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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301-363-4651 (Available 9 a.m. to 5 p.m. CST from Monday to Friday)
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Address
7505 Fannin St., Ste 610, Room 7 (CUBIO Innovation Center), Houston, TX 77054, USA
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