GAA Antikörper (N-Term)

Produktnummer ABIN8101555

Dieser Kaninchen Polyklonal Antikörper detektiert spezifisch GAA in WB. Es zeigt Reaktivität gegenüber Proben von Human.

Kurzübersicht für GAA Antikörper (N-Term) (ABIN8101555)

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

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser GAA Antikörper ist unkonjugiert

Applikation: Western Blotting (WB)

Produktdetails anti-GAA Antikörper

Bindungsspezifität: N-Term

Verwendungszweck: GAA Antibody - N-terminal region

Sequenz: RDLAPTPGAN LYGSHPFYLA LEDGGSAHGV FLLNSNAMDV VLQPSPALSW

Homologie: Dog: 86%, Guinea Pig: 100%, Horse: 86%, Human: 100%, Mouse: 100%, Rabbit: 100%, Rat: 100%

Aufreinigung: Affinity purified

Immunogen: The immunogen is a synthetic peptide directed towards the N terminal region of human GAA

Anwendungsinformationen

Applikationshinweise: Optimal working dilution should be determined by the investigator.

Kommentare:

We also sell a specific blocking peptide that can be used in combination with this antibody. You can find the blocking peptide under AAP44227-100UG

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 0.5 mg/mL

Buffer: Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.

Konservierungsmittel: Sodium azide

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

Handhabung: prevent freeze-thaw cycles

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: For short term use, store at 2-8C up to 1 week. For long term storage, store at -20 °C in small aliquots to prevent freeze-thaw cycles.

Details zu GAA

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

Andere Bezeichnung: GAA

Hintergrund:

Background Information: This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. 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. Alternative splicing results in multiple transcript variants.

Gene Name: glucosidase, alpha, acid

Alternative Symbols: LYAG

Protein Name: lysosomal alpha-glucosidase

Molekulargewicht: 104 kDa

Gen-ID: 2548

NCBI Accession: NP_000143

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process