G4046

Anti-Glucocerebrosidase antibody produced in rabbit

~1 mg/mL, affinity isolated antibody, buffered aqueous solution

• Sinónimos: Anti-D-glucosyl-N-acylsphingosine glucohydrolase, Anti-GBA, Anti-GBA1, Anti-Glucosidase, beta (Gluc), Anti-Glucosylceramidase (GlcCerase), Anti-Lysosomal glucocerebrosidase
• Código UNSPSC: 12352203
• NACRES: NA.41

Propiedades

• origen biológico: rabbit
• conjugado: unconjugated
• forma del anticuerpo: affinity isolated antibody
• tipo de anticuerpo: primary antibodies
• clon: polyclonal
• formulario: buffered aqueous solution
• mol peso: antigen ~60 kDa
• reactividad de especies: mouse, human, rat
• validación mejorada: recombinant expression; Learn more about Antibody Enhanced Validation
• concentración: ~1 mg/mL
• técnicas: western blot: 1-2 μg/mL using HEK-293T cell lysate expressing human glucocerebrosidase (GBA) or SH-SY-5Y cell lysate
• Nº de acceso UniProt: P04062
• Condiciones de envío: dry ice
• temp. de almacenamiento: −20°C
• modificación del objetivo postraduccional: unmodified
• Información sobre el gen: human ... GBA(2629) ; mouse ... Gba(14466)

Descripción

Descripción general

GBA1 (β-glucocerebrosidase) gene is mapped to human chromosome 1q21. It is a lysosomal enzyme and is widely expressed. The protein has domain I with three stranded anti?parallel βsheets, domain II with two β sheets making an immunoglobulin like domain and domain III with eight stranded β/α triosephosphate isomerase (TIM) barrel.

Aplicación

Anti-Glucocerebrosidase antibody produced in rabbit has been used in western blotting and immunocytochemistry.

Anti-Glucocerebrosidase antibody produced in rabbit is suitable for immunoblotting at a working concentration of 1-2μg/mL using HEK-293T cell lysate expressing human glucocerebrosidase (GBA).

Acciones bioquímicas o fisiológicas

GBA (glucosidase, beta, acid) gene encodes a protein that cleaves the β-glucosidic linkage of glycosylceramide (GlcCer), an intermediate in glycolipid metabolism. Mutations in the human GBA gene cause a reduction in the GBA activity and accumulation of GlcCer in lysosomes of cells of the reticuloendothelial system. This causes Gaucher disease (GD), an inherited lysosomal storage disorder, characterised by severe loss of neurons in the central nervous system, fetal onset, hydrops fetalis, in utero fetal death and neonatal distress. It leads to changes in neuronal functionality including increased levels of tubular endoplasmic reticulum (ER) elements, a large increase in Ca²⁺ release from the ER in response to glutamate, and an increased sensitivity to glutamate-induced neurotoxicity. Mutations in the human GBA gene may contribute to the development of common age-related dementia known as dementia with Lewy bodies or DLB and may cause early-onset of Parkinson disease.

Forma física

Solution in 0.01 M phos­phate buffered saline, pH 7.4, containing 15 mM sodium azide.

Cláusula de descargo de responsabilidad

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Información de seguridad

• Código de clase de almacenamiento: 12 - Non Combustible Liquids
• Clase de riesgo para el agua (WGK): WGK 1
• Punto de inflamabilidad (°F): Not applicable
• Punto de inflamabilidad (°C): Not applicable
• Equipo de protección personal: Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)