HIBCH Antibody (E-11): sc-515355

HIBCH Antibody (E-11) is a mouse monoclonal IgM antibody that detects HIBCH protein of mouse, rat, and human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). Anti-HIBCH antibody (E-11) is available as the non-conjugated form. HIBCH, also known as 3-hydroxyisobutyryl-CoA hydrolase, consists of 386 amino acids and belongs to the enoyl-CoA hydratase/isomerase family. HIBCH is primarily localized in the mitochondria, where HIBCH plays a crucial role in the metabolism of branched-chain fatty acids and certain amino acids. This localization is significant because mitochondrial dysfunction can lead to various metabolic disorders, including those associated with energy production and fatty acid metabolism. HIBCH is highly expressed in the liver and kidney, with lower expression levels in the heart, muscle, and brain. HIBCH hydrolyzes HIBYL-CoA, a saline catabolite, and beta-hydroxypropionyl-CoA, which is an intermediate in the minor pathway of propionate metabolism. The gene encoding HIBCH is located on human chromosome 2q32.2 and exists in two alternatively spliced isoforms. Defects in this gene can lead to HIBCH deficiency (HIBCHD), also known as deficiency of beta-hydroxyisobutyryl CoA deacylase or methacrylic aciduria, which is characterized by the accumulation of methacrylyl-CoA, a highly reactive compound that can interact with free sulfhydryl groups. Phenotypic symptoms of HIBCHD include early deterioration of neurological function, delayed motor skill development, and hypotonia, highlighting the importance of HIBCH in maintaining metabolic balance and neurological health.

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HIBCH Antibody (E-11) References:

1. Generation and annotation of the DNA sequences of human chromosomes 2 and 4.  |  Hillier, LW., et al. 2005. Nature. 434: 724-31. PMID: 15815621
2. Mutations in the gene encoding 3-hydroxyisobutyryl-CoA hydrolase results in progressive infantile neurodegeneration.  |  Loupatty, FJ., et al. 2007. Am J Hum Genet. 80: 195-9. PMID: 17160907
3. Lysine acetylation targets protein complexes and co-regulates major cellular functions.  |  Choudhary, C., et al. 2009. Science. 325: 834-40. PMID: 19608861
4. Hemizygous deletion of COL3A1, COL5A2, and MSTN causes a complex phenotype with aortic dissection: a lesson for and from true haploinsufficiency.  |  Meienberg, J., et al. 2010. Eur J Hum Genet. 18: 1315-21. PMID: 20648054
5. beta-hydroxyisobutyryl coenzyme A deacylase deficiency: a defect in valine metabolism associated with physical malformations.  |  Brown, GK., et al. 1982. Pediatrics. 70: 532-8. PMID: 7122152
6. Primary structure and tissue-specific expression of human beta-hydroxyisobutyryl-coenzyme A hydrolase.  |  Hawes, JW., et al. 1996. J Biol Chem. 271: 26430-4. PMID: 8824301

Monoclonal antibodies for mammalian and non-mammalian protein targets represent essentially all targets covered by polyclonal antibodies. Over 8,300 of our antibodies are offered as ImmunoCruz^® conjugates which eliminates the need for a secondary antibody.

Santa Cruz Biotechnology antibodies have over 360,000 research citations. Anti-HIBCH Antibody (E-11) has 3 citations in a variety of scientific publications.

Primary antibodies like Anti-HIBCH Antibody (E-11) for mammalian target proteins are recommended for the detection of a range of mammalian species, primarily of mouse, rat and human species. Many mammalian antibodies are also ideal for veterinary research and reactive with ovine, porcine, caprine, feline, canine, bovine and equine protein targets. Santa Cruz Biotechnology primary monoclonal antibodies are suitable for laboratory research applications and are not intended for diagnostic or therapeutic use.

HIBCH Antibody (E-11) Specifications

Alternate Product Name: HIBCH

Product Clone Name: E-11

Classification: Primary Antibody

Clonality: Monoclonal Antibody

Antibody Isotype: IgM kappa light chain

Amino Acids: 272-296

Epitope Target: HIBCH

Epitope Species: human

Product Reactivity/Detection: HIBCH

Species Reactivity/Detection: mouse, rat, human

Additional Mammalian Reactivity: N/A

Additional Non-Mammalian Reactivity: N/A

Applications: WB, IP, IF, IHC(P), ELISA

Vialing Information: Each vial contains 200 µg IgM kappa light chain in 1.0 ml of PBS with < 0.1% sodium azide and 0.1% gelatin

Research Citations: 3 citations in various scientific publications

Available Conjugates/Forms for HIBCH Antibody (E-11): P: Blocking Peptide