Rabbit anti-Human GLA  Monoclonal Antibody | anti-GLA  antibody

Rabbit anti Human GLA  Monoclonal Antibody

Cat. Num. AAA2544332

• Gene Names: NAT8; GLA; CML1; CCNAT; Hcml1; ATase2; TSC501; TSC510
• Reactivity: Human
• Applications: Western Blot, ELISA, Immunoprecipitation
• Synonyms: GLA ; Monoclonal Antibody; Rabbit anti Human GLA Monoclonal Antibody; anti-GLA antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG
• Sequence Length: 227

• Applicable Applications for anti-GLA antibody: Western Blot (WB), ELISA (EIA), Immunoprecipitation (IP)
• Application Notes: WB: 0.5-2 mug/mL; ELISA: 0.1-0.2 mug/mL. This antibody can be used at 0.1-0.2 mug/mL with the appropriate secondary reagents to detect Human GLA. The detection limit for Human GLA is approximately 0.0195 ng/well.; IP: 4-6 mug/mg of lysate
• Immunogen: Recombinant Human alpha-Galactosidase A/GLA protein
• Buffer: 0.2 mum filtered solution in PBS with 5% trehalose
• Preparation and Storage: This antibody can be stored at 2 degree C- 8 degree C for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20 degree C to -80 degree C. Preservative-Free. Sodium azide is recommended to avoid contamination (final concentration 0.05%-0.1%). It is toxic to cells and should be disposed of properly. Avoid repeated freeze-thaw cycles.

Related Product Information for anti-GLA  antibody

Alpha-galactosidase A, also known as Alpha-D-galactoside galactohydrolase, Alpha-D-galactosidase A, Melibiase and GLA, is a member of the glycosyl hydrolase 27 family. GLA is used as a long-term enzyme replacement therapy in patients with a confirmed identification of Fabry disease. Defects in GLA are the cause of Fabry disease (FD) which is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Deficiency of GLA leads to the accumulation of glycosphingolipids in the vasculature leading to multiorgan pathology. In addition to well-described microvascular disease, deficiency of GLA is also characterized by premature macrovascular events such as stroke and possibly myocardial infarction.

NCBI and Uniprot Product Information

• NCBI GI #: 25294240
• NCBI GeneID: 9027
• Molecular Weight: 25,619 Da
• NCBI Official Full Name: GLA
• NCBI Official Synonym Full Names: N-acetyltransferase 8 (putative)
• NCBI Official Symbol: NAT8
• NCBI Official Synonym Symbols: GLA; CML1; CCNAT; Hcml1; ATase2; TSC501; TSC510
• NCBI Protein Information: N-acetyltransferase 8
• UniProt Protein Name: N-acetyltransferase 8
• UniProt Gene Name: NAT8
• UniProt Synonym Gene Names: ATase2; CCNAT
• UniProt Entry Name: NAT8_HUMAN

NCBI Description

This gene, isolated using the differential display method to detect tissue-specific genes, is specifically expressed in kidney and liver. The encoded protein shows amino acid sequence similarity to N-acetyltransferases. A similar protein in Xenopus affects cell adhesion and gastrulation movements, and may be localized in the secretory pathway. A highly similar paralog is found in a cluster with this gene. [provided by RefSeq, Sep 2008]

Uniprot Description

NAT8: Plays a role in regulation of gastrulation. Belongs to the camello family.

Protein type: Acetyltransferase; Cell adhesion; EC 2.3.1.80; Membrane protein, integral; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: 2p13.2

Cellular Component: endoplasmic reticulum membrane; ER-Golgi intermediate compartment; ER-Golgi intermediate compartment membrane; integral to membrane

Molecular Function: cysteine-S-conjugate N-acetyltransferase activity; lysine N-acetyltransferase activity; protein binding

Biological Process: beta-amyloid metabolic process; cellular protein metabolic process; glutathione metabolic process; negative regulation of apoptosis; peptidyl-lysine N6-acetylation; response to drug