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HEXB Rabbit anti-Human, Mouse, Rat, Polyclonal, Proteintech
Rabbit Polyclonal Antibody
140.00 € - 399.00 €
Spezifikation
Antigen | HEXB |
---|---|
Konzentration | 0.35 mg/mL |
Anwendungen | Immunohistochemistry (Paraffin), Western Blot |
Klassifikation | Polyclonal |
Konjugat | Unconjugated |
Produktcode | Marke | Menge | Preis | Menge & Verfügbarkeit | |||||
---|---|---|---|---|---|---|---|---|---|
Produktcode | Marke | Menge | Preis | Menge & Verfügbarkeit | |||||
16895784
|
Proteintech
16229-1-AP-20UL |
20 μL |
140.00 €
20 Mikroliter |
Verfügbar ab: 29-04-2024 Loggen Sie sich ein, um den Lagerbestand zu sehen |
Bitte melden Sie sich an, um diesen Artikel zu kaufen. Sie benötigen ein Web-Konto? Registrieren Sie sich noch heute! | ||||
16885784
|
Proteintech
16229-1-AP-150UL |
150 μL |
399.00 €
150 Mikroliter |
Verfügbar ab: 29-04-2024 Loggen Sie sich ein, um den Lagerbestand zu sehen |
Bitte melden Sie sich an, um diesen Artikel zu kaufen. Sie benötigen ein Web-Konto? Registrieren Sie sich noch heute! | ||||
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
HEXB | |
Immunohistochemistry (Paraffin), Western Blot | |
Unconjugated | |
Rabbit | |
Human, Mouse, Rat | |
P07686, P20060, Q6AXR4 | |
15212, 294673, 3074 | |
HEXB Fusion Protein Ag8683 | |
Primary | |
-20°C | |
HEXB |
0.35 mg/mL | |
Polyclonal | |
Liquid | |
RUO | |
PBS with 50% glycerol and 0.02% sodium azide; pH 7.3 | |
ENC 1AS, HCC 7, HEXB, Hexosaminidase subunit B | |
Hexb | |
IgG | |
Antigen Affinity Chromatography | |
Antibody |