HEXB anticorps

N° du produit ABIN7861437

L’anticorps anti-HEXB Monoclonal Souris est utilisé pour la détection de HEXB dans des échantillons de Humain. Il a été validé pour IHC.

Aperçu rapide pour HEXB anticorps (ABIN7861437)

Antigène: HEXB (Hexosaminidase B (Beta Polypeptide) (HEXB))

Reactivité: Humain

Hôte: Souris

Clonalité: Monoclonal

Conjugué: Cet anticorp HEXB est non-conjugé

Application: Immunohistochemistry (IHC)

Clone: HEXB-7762

Détail du produit anti-HEXB anticorps

Fonction: HEXB Antibody

Specificité: Hexosaminidase B (HEXB), also designated ≤-hexosaminidase B, is a hexosaminidase B (HEXB), also designated ≤-hexosaminidase B, is a tetramer of two ≤-A and two ≤-B chains and is found in the lysosomes of cells. Sandhoff disease (SD), also known as GM2-gangliosidosis type II, is caused by mutations in the HEXB gene that affect the ≤ subunit. These mutations disrupt the activity of HEXB and HEXA, which prevents the breakdown of GM2 ganglioside, a fatty material found in the brain, therby rendering both the HEXA and HEXB enzymes deficient. SD is a rare autosomal recessive disorder characterized by an accumulation of GM2 ganglioside, which causes progressive destruction of the central nervous system. Sandhoff disease is similar to Tay-Sachs disease, which is caused by mutations in the HEXA gene, although SD is more severe.

Purification: 200ug/ml of Ab purified from Bioreactor Concentrate by Protein A/G.

Immunogène: Recombinant fragment human HEXB protein (exact sequence is proprietary)

Isotype: IgG2, kappa

Information d'application

Indications d'application: Optimal working dilution should be determined by the investigator.

Restrictions: For Research Use only

Stockage

Concentration: 1.0 mg/mL

Buffer: Prepared in 10  mM PBS

Agent conservateur: Azide free

Stock: -20 °C,-80 °C

Stockage commentaire: Antibody with azide - store at 2 to 8°C. Antibody without azide - store at -20 to -80°C.Antibody is stable for 24 months. Non-hazardous. No MSDS required.

Détails sur HEXB

Antigène: HEXB (Hexosaminidase B (Beta Polypeptide) (HEXB))

Autre désignation: HEXB

Sujet:

Beta N acetylhexosaminidase, Cervical cancer proto oncogene 7 protein, ENC 1AS, Epididymis luminal protein 248, HCC7, HEL 248, Hexosaminidase B (beta polypeptide), Hexosaminidase B, N-acetyl-beta-glucosaminidase subunit beta

Human kidney or lung.

Poids moléculaire: 63kDa

ID gène: 3074, 69293

Pathways: Sensory Perception of Sound, Glycosaminoglycan Metabolic Process