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HEXB Rabbit anti-Human, Mouse, Rat, Polyclonal, Proteintech

Rabbit Polyclonal Antibody

Marke:  Proteintech 16229-1-AP-150UL

Weitere Details : Netto-Gewicht : 0.00010kg

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Artikelnummer. 16885784

  • 329.85 € / 150 Mikroliter

Voraussichtliches Versanddatum: 25-06-2021

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Beschreibung

Beschreibung

Hexosaminidase B (HEXB), also designated beta-hexosaminidase B, is a Hexosaminidase B (HEXB), also designated b-hexosaminidase B, is a tetramer of two b-A and two b-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 b 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.
Spezifikation

Spezifikation

HEXB
0.35 mg/mL
PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
P07686, P20060, Q6AXR4
ENC 1AS, HCC 7, HEXB, Hexosaminidase subunit B
Rabbit
IgG
150 μL
-20°C
Primary
15212, 294673, 3074
Immunohistochemistry (Paraffin), Western Blot
Unconjugated
HEXB
Liquid
Hexb
HEXB Fusion Protein Ag8683
Antigen Affinity Chromatography
RUO
Antibody
Polyclonal
Human, Mouse, Rat
Videos
Sicherheitsdatenblatt (SDS)
Dokumentation
Zertifikate