GAA Antikörper (N-Term)

Produktnummer ABIN656475

Produktdetails anti-GAA Antikörper

Target: GAA (Glucosidase, Alpha, Acid (GAA))

Bindungsspezifität: AA 174-203, N-Term

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser GAA Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), Immunohistochemistry (IHC), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Aufreinigung: This antibody is purified through a protein A column, followed by peptide affinity purification.

Immunogen: This GAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 174-203 amino acids from the N-terminal region of human GAA.

Klon: RB18979

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: WB: 1:2000. WB: 1:1000. WB: 1:1000. WB: 1:1000. IHC-P-Leica: 1:500. IHC-P-Leica: 1:500

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Buffer: Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.

Konservierungsmittel: Sodium azide

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

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: GAA Antibody (N-term) can be refrigerated at 2-8 °C for up to 6 months. For long term storage, place the at -20 °C.

Haltbarkeit: 6 months

Details zu GAA

Target: GAA (Glucosidase, Alpha, Acid (GAA))

Andere Bezeichnung: GAA (GAA Produkte)

Synonyme: LYAG antikoerper, E430018M07Rik antikoerper, glucosidase alpha, acid antikoerper, glucosidase, alpha, acid antikoerper, transmembrane and coiled-coil domain family 1 antikoerper, glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II) antikoerper, GAA antikoerper, Gaa antikoerper, TMCC1 antikoerper, gaa antikoerper

Hintergrund: This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene.

Molekulargewicht: 105324

Gen-ID: 2548

NCBI Accession: NP_000143, NP_001073271, NP_001073272

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process