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Products:Tags & Cell Markers >> Subcellular Markers >> Organelles >> Lysosome
Overview
Product name
Anti-HEXA antibody
See all HEXA products (3) ...
Description
Mouse polyclonal to HEXA
Tested applications
Cross reactivity
Reacts with
Human
Immunogen
Full length human HEXA protein (NP_000511.1)
Positive control
HEXA transfected 293T cell lysate.
Properties
Form
Liquid
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Storage buffer
Preservative: None
Constituents: 1X PBS, pH 7.2
Concentration
Concentration information loading...
Purity
Protein G purified
Clonality
Polyclonal
Isotype
IgG
Research areas
Neuroscience >> Neurology process >> Neurogenesis
Neuroscience >> Neurology process >> Neurodegenerative disease >> Other
Tags & Cell Markers >> Subcellular Markers >> Organelles >> Lysosome
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Western blot - Anti-HEXA antibody (ab70083)
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Applications
Show applications key
Our Abpromise guarantee covers the use of ab70083 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
WB: 1/500 - 1/1000.Detects a band of approximately 61 kDa (predicted molecular weight: 61 kDa).
Target
Function
Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. The form B is active against certain oligosaccharides. The form S has no measurable activity.
Involvement in disease
Defects in HEXA are the cause of GM2-gangliosidosis type 1 (GM2G1) [MIM:272800]; also known as Tay-Sachs disease. GM2-gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. GM2G1 is characterized by GM2 gangliosides accumulation in the absence of HEXA activity, leading to neurodegeneration and, in the infantile form, death in early childhood. GM2G1 has an increased incidence among Ashkenazi Jews and French Canadians in eastern Quebec. It exists in several forms: infantile (most common and most severe), juvenile and adult (late onset).
Sequence similarities
Belongs to the glycosyl hydrolase 20 family.
Post-translational
modifications
N-linked glycan at Asn-115 consists of Man(3)-GlcNAc(2).
Cellular localization
Lysosome.
Target information above from: UniProt accessionP06865
The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010).
Alternative names
- Beta hexosaminidase alpha chain precursor antibody
- Beta hexosaminidase subunit alpha antibody
- Beta N acetylhexosaminidase antibody
see all
Database links
- Entrez Gene: 3073 Human
- Omim: 606869 Human
- SwissProt: P06865 Human
- Unigene: 604479 Human
- Unigene: 709495 Human
Anti-HEXA antibody images:
Western blot - Anti-HEXA antibody (ab70083)
All lanes : Anti-HEXA antibody (ab70083) at 1/500 dilution
Lane 1 : HEXA transfected 293T cell lysate
Lane 2 : Non-transfected 293T cell lysate
Lysates/proteins at 25 µg per lane.
Secondary
Goat Anti-Mouse IgG (H&L)-HRP Conjugate at 1/2500 dilution
Predicted band size : 61 kDa
Observed band size : 61 kDa
Blocking in 5% milk in PBST.
References for Anti-HEXA antibody (ab70083)
ab70083 has not yet been referenced specifically in any publications.
Publishing research using ab70083? Please let us know so that we can cite the reference in this datasheet
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"