Antibody data
- Antibody Data
- Antigen structure
- References [0]
- Comments [0]
- Validations
- Western blot [1]
Submit
Validation data
Reference
Comment
Report error
- Product number
- BS1917 - Provider product page
- Provider
- Bioworld Technology, Inc
- Product name
- Hexb (S516) polyclonal antibody
- Antibody type
- Polyclonal
- Antigen
- Synthetic peptide, corresponding to amino acids 481-530 of Human Hexb.
- Description
- 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.
- Reactivity
- Human, Mouse, Rat
- Host
- Rabbit
- Isotype
- IgG
- Vial size
- 100ul
- Concentration
- 1 mg/ml
- Storage
- Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
No comments: Submit comment
Supportive validation
- Submitted by
- Bioworld Technology, Inc (provider)
- Main image
- Experimental details
- Western blot (WB) analysis of Hexb (S516) pAb at 1:500 dilutionLane1:Jurkat whole cell lysateLane2:Raw264.7 whole cell lysateLane3:H9C2 whole cell lysate