HIBCH Antikörper (AA 251-350) (AbBy Fluor® 647)

Produktnummer ABIN1698222

Produktdetails anti-HIBCH Antikörper (AbBy Fluor® 647)

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

Bindungsspezifität: AA 251-350

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser HIBCH Antikörper ist konjugiert mit AbBy Fluor® 647

Applikation: Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Homologie: Human

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human HIBCH

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Konservierungsmittel: ProClin

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

Lagerung: -20 °C

Informationen zur Lagerung: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Haltbarkeit: 12 months

Details zu HIBCH

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

Andere Bezeichnung: Hibch (HIBCH Produkte)

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

Hintergrund:

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.

Gen-ID: 26275

Pathways: Monocarboxylic Acid Catabolic Process