HIBCH antibody (AA 251-350)

Catalog No. ABIN1713537

Product Details anti-HIBCH Antibody

Target: HIBCH (3-hydroxyisobutyryl-CoA Hydrolase (HIBCH))

Binding Specificity: AA 251-350

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This HIBCH antibody is un-conjugated

Application: Western Blotting (WB), ELISA, Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunocytochemistry (ICC), Immunohistochemistry (Frozen Sections) (IHC (fro))

Predicted Reactivity: Human

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human HIBCH

Isotype: IgG

Application Details

Application Notes: WB 1:300-5000
ELISA 1:500-1000
IHC-P 1:200-400
IHC-F 1:100-500
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200
ICC 1:100-500

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Storage: 4 °C,-20 °C

Storage Comment: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Expiry Date: 12 months

Target Details for HIBCH

Target: HIBCH (3-hydroxyisobutyryl-CoA Hydrolase (HIBCH))

Alternative Name: HIBCH (HIBCH Products)

Synonyms: DDBDRAFT_0189353 antibody, DDBDRAFT_0233831 antibody, DDB_0189353 antibody, DDB_0233831 antibody, T11J7.4 antibody, T11J7_4 antibody, T11J7.5 antibody, T11J7_5 antibody, HIBYLCOAH antibody, 2610509I15Rik antibody, AI648812 antibody, zgc:110824 antibody, 3-hydroxyisobutyryl-coenzyme A hydrolase antibody, 3-hydroxyisobutyryl-coenzyme a hydrolase,putative antibody, 3-hydroxyisobutyryl-Coenzyme A hydrolase antibody, 3-hydroxyisobutyryl-CoA hydrolase (predicted) antibody, 3-hydroxyisobutyryl-CoA hydrolase antibody, ATP-dependent caseinolytic (Clp) protease/crotonase family protein antibody, putative 3-hydroxyisobutyryl-coenzyme A hydrolase antibody, 3-hydroxyisobutyryl-CoA hydrolase L homeolog antibody, PVX_100950 antibody, CpipJ_CPIJ012030 antibody, PKH_145800 antibody, hibch antibody, MCYG_05070 antibody, SPBC2D10.09 antibody, CNA02940 antibody, LACBIDRAFT_292320 antibody, SJAG_00944 antibody, VDBG_07520 antibody, AT2G30650 antibody, AT2G30660 antibody, LBRM_32_3920 antibody, HIBCH antibody, Hibch antibody, hibch.L antibody

Background:

Synonyms: 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, HIBCH_HUMAN.

Background: HIBCH is a 386 amino acid protein belonging to the enoyl-CoA hydratase/isomerase family. Localizing to the mitochondria, HIBCH is highly expressed in liver and kidney, with lower levels found in heart, muscle and brain. HIBCH hydrolyzes HIBYL-CoA, a saline catabolite, and _-hydroxypropionyl-CoA, an intermediate in the minor pathway involved in the metabolism of proprionate. Existing as two alternatively spliced isoforms, the gene encoding HIBCH maps to human chromosome 2q32.2. Defects to this gene result in HIBCH deficiency (HIBCHD), known alternatively as deficiency of _-hydroxyisobutyryl CoA deacylase or methacrylic aciduria. HIBCHD is characterized by the accumulation of methacrylyl-CoA, a highly reactive compound that undergoes addition reactions with free sulfhydryl groups. Phenotypic symptoms include early deterioration of neurological function, delayed motor skill development and hypotonia.

Gene ID: 26275

Pathways: Monocarboxylic Acid Catabolic Process