alpha-Galactosidase A / GLA Recombinant Protein | GLA recombinant protein

Recombinant Human alpha-Galactosidase A / GLA Protein (His tag)

Cat. Num. AAA2545582

• Gene Names: PRLR; HPRL; MFAB; hPRLrI
• Purity: > 97 % as determined by SDS-PAGE
• Synonyms: alpha-Galactosidase A / GLA; Recombinant Human alpha-Galactosidase A / GLA Protein (His tag); GALA; GLA recombinant protein

• Host: Human Cells
• Purity/Purification: > 97 % as determined by SDS-PAGE
• Form/Format: Lyophilized from sterile 50mM Tris, 150mM NaCl, pH 7.5
• Sequence Length: 622

• Application Notes: The secreted recombinant human GLA consists of 409 amino acids and has a predicted molecular mass of 46.8 kDa. In SDS-PAGE under reducing conditions, it migrates as an approximately 50 kDa band.
• Predicted N Terminal: Leu 32
• Endotoxin: < 1.0 EU per mug of the protein as determined by the LAL method
• Preparation and Storage: Samples are stable for up to twelve months from date of receipt at -70 degree C

SDS-PAGE

Related Product Information for GLA recombinant protein

Background: 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.

Description: A DNA sequence encoding the human GLA (NP_000940.1) (Met 1-Leu 429) was fused with a polyhistidine tag at the C-terminus.

NCBI and Uniprot Product Information

• NCBI GI #: 4506107
• NCBI GeneID: 5618
• NCBI Accession #: NP_000940.1
• NCBI GenBank Nucleotide #: NM_000949.6
• UniProt Accession #: P16471; Q16354; Q8TD75; Q8TD78; Q96P35; Q96P36; Q9BX87; Q9UHJ5; B2R882; D1MDP1
• Molecular Weight: 35,011 Da
• NCBI Official Full Name: prolactin receptor isoform 1
• NCBI Official Synonym Full Names: prolactin receptor
• NCBI Official Symbol: PRLR
• NCBI Official Synonym Symbols: HPRL; MFAB; hPRLrI
• NCBI Protein Information: prolactin receptor
• UniProt Protein Name: Prolactin receptor
• Protein Family: Alpha-galactosidase
• UniProt Gene Name: PRLR
• UniProt Synonym Gene Names: PRL-R

NCBI Description

This gene encodes a receptor for the anterior pituitary hormone, prolactin, and belongs to the type I cytokine receptor family. Prolactin-dependent signaling occurs as the result of ligand-induced dimerization of the prolactin receptor. Several alternatively spliced transcript variants encoding different membrane-bound and soluble isoforms have been described for this gene, which may function to modulate the endocrine and autocrine effects of prolactin in normal tissue and cancer. [provided by RefSeq, Feb 2011]

Uniprot Description

PRLR: receptor for the anterior pituitary hormone prolactin and placental lactogen I and II. Interacts with Cyclophilin A, differentially regulating various signaling pathways from the PrlR. Prolactin signaling is attenuated by threonine and tyrosine phosphorylation. Two alternative splice isoforms have been identified.

Protein type: Membrane protein, integral; Receptor, cytokine

Chromosomal Location of Human Ortholog: 5p13.2

Cellular Component: cell surface; plasma membrane

Molecular Function: peptide hormone binding; protein binding

Biological Process: embryo implantation; transmembrane receptor protein tyrosine kinase activation (dimerization)

Disease: Hyperprolactinemia; Multiple Fibroadenomas Of The Breast