Product Name: HEXA/Beta hexosaminidase subunit alpha Antibody, ALEXA FLUOR® 647 Conjugated
Applications: IF(IHC-P)
Reactivity: Human, Mouse, Rat
Conjugation: ALEXA FLUOR® 647
Host: Rabbit
Sourcr: KLH conjugated synthetic peptide derived from human Beta hexosaminidase subunit alpha
Clonality: Polyclonal
CAS NO: 159351-69-6
product targets : HIV Integrase inhibitors
Isotype: IgG
Concentration: 1ug/ul
Purification: Purified by Protein A.
Storage: Aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Store at 4°C for 12 months.
Synonyms: Beta hexosaminidase alpha chain precursor; Beta hexosaminidase subunit alpha; Beta N acetylhexosaminidase; Beta N acetylhexosaminidase subunit alpha; Beta-hexosaminidase subunit alpha; Beta-N-acetylhexosaminidase subunit alpha; Hexa; HEXA_HUMAN; Hexosaminidase A alpha polypeptide; Hexosaminidase A alpha polypeptide; Hexosaminidase A; Hexosaminidase subunit A; MGC99608; N acetyl beta glucosaminidase; N acetyl beta glucosaminidase subunit alpha; N-acetyl-beta-glucosaminidase subunit alpha; TSD.
Background: Hexosaminidase A (HEXA), also designated beta-Hexosaminidase A, is a trimer composed of one å chain, one ∫-A chain and one ∫-B chain and is found in the lysosomes of cells. HEXA, along with the cofactor CM2 activator protein, catalyzes the degradation of GM2 ganglioside and other molecules containing terminal N-acetyl hexosamines in the brain and other tissues. A mutation in the å subunit of hexosaminidase is the cause of Tay-Sachs disease (TSD), also known as GM2-gangliosidosis type I. TSD is a fatal autosomal recessive lysosomal storage disease of the central nervous system (CNS) caused by insufficient activity of the HEXA enzyme that results in a failure to process GM2 gangliosides. The accumulation of GM2 ganglioside in the absence of HEXA activity causes progressive destruction of the CNS.
PubMed ID:http://www.ncbi.nlm.nih.gov/pubmed/17194893?dopt=Abstract
