LAMP2 recombinant protein

LAMP2 Protein, Human, Recombinant (His Tag)

Cat. Num. AAA8121464

• Gene Names: LAMP2; LAMPB; CD107b; LAMP-2; LGP110
• Purity: >95% as determined by SDS-PAGE
• Synonyms: LAMP2; LAMP2 Protein; Human; Recombinant (His Tag); Human LAMP2/CD107b Protein (His Tag); CD107b Protein; LAMP-2 Protein; LAMPB Protein; LGP110 Protein; lysosomal-associated membrane protein 2; LAMP2 recombinant protein

• Host: HEK293 Cells
• Purity/Purification: >95% as determined by SDS-PAGE
• Form/Format: Lyophilized from sterile PBS, pH7.4. Normally 5%-8% trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization. Please refer to the specific buffer information in the hard copy of CoA.
• Sequence: Met1-Ile375

• Species: Human
• Endotoxin: <1.0EU per ug of the protein as determined by the LAL method
• Predicted N Terminal: Leu 29
• Tag: C-His
• Protein Construction: A DNA sequence encoding the human LAMP2 (NP_054701.1)(Met1-Ile375) was expressed with a C-terminal polyhistidine tag.
• Reconstitution: A hardcopy of COA with reconstitution instruction is sent along with the products. Please refer to it for detailed information.
• Preparation and Storage: Samples are stable for up to twelve months from date of receipt at -20 degree C to -80 degree C. Store it under sterile conditions at -20 degree C to -80 degree C. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.; In general, recombinant proteins are provided as lyophilized powder which are shipped at ambient temperature. Bulk packages of recombinant proteins are provided as frozen liquid.; They are shipped out with blue ice.

SDS-Page

Related Product Information for LAMP2 recombinant protein

Background: LAMP2 (Lysosomal Associated Membrane Protein 2) is a Protein Coding gene. LAMP2, also known as CD107b (Cluster of Differentiation 107b), is a member of a family of membrane glycoproteins. This glycoprotein provides selectins with carbohydrate ligands. It may play a role in tumor cell metastasis. It may also function in the protection, maintenance, and adhesion of the lysosome. In humans, LAMP2, the causative gene of Danon disease, located on chromosome Xq24, encodes the lysosome-associated membrane protein-2 (LAMP-2). LAMP-2 deficiency, or Danon disease, is a rare X-linked lysosomal disease characterized by cardiomyopathy, vacuolar myopathy, and mental retardation. LAMP2 cardiomyopathy is an X-linked and highly progressive myocardial storage disorder associated with diminished survival, which clinically resembles sarcomeric hypertrophic cardiomyopathy.

References

Maron BJ, et al. (2010) Profound left ventricular remodeling associated with LAMP2 cardiomyopathy. Am J Cardiol. 106(8): 1194-6.Di Blasi C, et al. (2008) Danon disease: a novel LAMP2 mutation affecting the pre-mRNA splicing and causing aberrant transcripts and partial protein expression. Neuromuscul Disord. 18(12): 962-6.Echaniz-Laguna A, et al. (2006) Novel Lamp-2 gene mutation and successful treatment with heart transplantation in a large family with Danon disease. Muscle Nerve. 33(3): 393-7.

NCBI and Uniprot Product Information

• NCBI GI #: 7669503
• NCBI GeneID: 3920
• NCBI Accession #: NP_054701.1
• NCBI GenBank Nucleotide #: NM_013995.2
• UniProt Accession #: P13473; Q16641; Q6Q3G8; Q96J30; Q99534; Q9UD93; A8K4X5; D3DTF0
• Molecular Weight: 45,170 Da
• NCBI Official Full Name: lysosome-associated membrane glycoprotein 2 isoform B
• NCBI Official Synonym Full Names: lysosomal-associated membrane protein 2
• NCBI Official Symbol: LAMP2
• NCBI Official Synonym Symbols: LAMPB; CD107b; LAMP-2; LGP110
• NCBI Protein Information: lysosome-associated membrane glycoprotein 2; CD107 antigen-like family member B
• UniProt Protein Name: Lysosome-associated membrane glycoprotein 2
• Protein Family: Lysosome-associated membrane glycoprotein
• UniProt Gene Name: LAMP2
• UniProt Synonym Gene Names: LAMP-2; Lysosome-associated membrane protein 2
• UniProt Entry Name: LAMP2_HUMAN

NCBI Description

The protein encoded by this gene is a member of a family of membrane glycoproteins. This glycoprotein provides selectins with carbohydrate ligands. It may play a role in tumor cell metastasis. It may also function in the protection, maintenance, and adhesion of the lysosome. Alternative splicing of this gene results in multiple transcript variants encoding distinct proteins. [provided by RefSeq, Jul 2008]

Uniprot Description

LAMP2: Implicated in tumor cell metastasis. May function in protection of the lysosomal membrane from autodigestion, maintenance of the acidic environment of the lysosome, adhesion when expressed on the cell surface (plasma membrane), and inter- and intracellular signal transduction. Protects cells from the toxic effects of methylating mutagens. Defects in LAMP2 are the cause of Danon disease (DAND); also known as glycogen storage disease type 2B (GSD2B). DAND is a lysosomal glycogen storage disease characterized by the clinical triad of cardiomyopathy, vacuolar myopathy and mental retardation. It is often associated with an accumulation of glycogen in muscle and lysosomes. Belongs to the LAMP family. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, integral

Chromosomal Location of Human Ortholog: Xq24

Cellular Component: phagocytic vesicle membrane; membrane; lysosome; lysosomal membrane; late endosome membrane; late endosome; plasma membrane; integral to membrane; platelet dense granule membrane

Molecular Function: protein domain specific binding; enzyme binding

Biological Process: platelet activation; platelet degranulation; protein stabilization; blood coagulation

Disease: Danon Disease