HIBCH Antikörper (AA 251-350) (Cy3)

Produktnummer ABIN1701848

Der Kaninchen Polyklonal Anti-HIBCH-Antikörper wurde für WB, IF (cc) und IF (p) validiert. Er ist geeignet, HIBCH in Proben von Human zu detektieren.

Kurzübersicht für HIBCH Antikörper (AA 251-350) (Cy3) (ABIN1701848)

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

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser HIBCH Antikörper ist konjugiert mit Cy3

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

Produktdetails anti-HIBCH Antikörper (Cy3)

Bindungsspezifität: AA 251-350

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

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