Rabbit anti-Human, Mouse Hexosaminidase A Polyclonal Antibody | anti-HEXA antibody

Hexosaminidase A. NT (Hexosaminidase Subunit A, HEXA, Beta-hexosaminidase Subunit alpha, Beta-N-acetylhexosaminidase Subunit alpha, MGC99608, N-acetyl-beta-glucosaminidase Subunit alpha, TSD) (Biotin)

Cat. Num. AAA6480285

• Gene Names: HEXA; TSD
• Reactivity: Human, Mouse
• Applications: Immunohistochemistry, ELISA, Western Blot, Flow Cytometry, Functional Assay
• Purity: Purified by Protein A and Peptide Affinity Chromatography.
• Synonyms: Hexosaminidase A; Polyclonal Antibody; Hexosaminidase A. NT (Hexosaminidase Subunit A; HEXA; Beta-hexosaminidase Subunit alpha; Beta-N-acetylhexosaminidase Subunit alpha; MGC99608; N-acetyl-beta-glucosaminidase Subunit alpha; TSD) (Biotin); Hexosaminidase A (alpha Polypeptide); EC=3.2.1.52; anti-HEXA antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human Hexosaminidase A. Species Crossreactivity: mouse
• Purity/Purification: Purified by Protein A and Peptide Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Biotin.

• Applicable Applications for anti-HEXA antibody: Immunohistochemistry (IHC) Paraffin, ELISA (EIA), Western Blot (WB), Flow Cytometry (FC/FACS)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 142-170 in the N-terminal region of human Hexosaminidase A. Swiss/UniProt Accession: P06865.
• Conjugate: Biotin
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blot analysis in HepG2 cell line lysates (35ug/lane) usingMBS624677. HEXA (arrow) was detected usingMBS624677.)

Western Blot (WB)

(Western Blot analysis in mouse liver tissue lysates (35ug/lane) usingMBS624677. HEXA (arrow) was detected usingMBS624677.)

Immunohistochemistry (IHC)

(Immunohistochemistry analysis in formalin-fixed and paraffin-embedded human brain tissue usingMBS624677, followed by peroxidase-conjugated to the secondary antibody and DAB staining. This data demonstrates the use of MBS624677 for immunohistochemistry.)

Flow Cytometry (FC/FACS)

(Flow Cytometric analysis of HepG2 cells using MBS624677 (right histogram) compared to a negative control cell (left histogram). FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)

Product Categories/Family for anti-HEXA antibody

Antibodies; Enzymes; Glycosidase

References

1. Park, N.J., et.al., Pediatr. Res. (2009). 2. Pennybacker, M., et.al., J. Biol. Chem. 271(29), 17377-17382 (1996).

NCBI and Uniprot Product Information

• NCBI GI #: 189181666
• NCBI GeneID: 3073
• NCBI Accession #: NP_000511.2
• NCBI GenBank Nucleotide #: NM_000520.5
• UniProt Accession #: P06865; Q53HS8; Q6AI32; B4DKE7; E7ENH7
• Molecular Weight: 19,326 Da
• NCBI Official Full Name: beta-hexosaminidase subunit alpha isoform 2 preproprotein
• NCBI Official Synonym Full Names: hexosaminidase subunit alpha
• NCBI Official Symbol: HEXA
• NCBI Official Synonym Symbols: TSD
• NCBI Protein Information: beta-hexosaminidase subunit alpha
• UniProt Protein Name: Beta-hexosaminidase subunit alpha
• Protein Family: Hexamerin
• UniProt Gene Name: HEXA
• UniProt Synonym Gene Names: Hexosaminidase subunit A
• UniProt Entry Name: HEXA_HUMAN

Uniprot Description

HEXA: 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. Defects in HEXA are the cause of GM2-gangliosidosis type 1 (GM2G1); 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). Belongs to the glycosyl hydrolase 20 family.

Protein type: Carbohydrate Metabolism - amino sugar and nucleotide sugar; EC 3.2.1.52; Glycan Metabolism - glycosaminoglycan degradation; Glycan Metabolism - glycosphingolipid biosynthesis - ganglio series; Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Glycan Metabolism - other glycan degradation; Hydrolase

Chromosomal Location of Human Ortholog: 15q24.1

Cellular Component: azurophil granule; lysosomal lumen; membrane

Molecular Function: acetylglucosaminyltransferase activity; beta-N-acetylhexosaminidase activity; protein heterodimerization activity

Biological Process: chondroitin sulfate catabolic process; glycosaminoglycan biosynthetic process; glycosphingolipid metabolic process; hyaluronan catabolic process; keratan sulfate catabolic process

Disease: Tay-sachs Disease