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Cynomolgus LAMP2 Protein (His Tag)

LAMP2

Catalog Number P90208-C08H
Organism Species Cynomolgus
Host Human Cells
Synonyms LAMP2
Molecular Weight The recombinant cynomolgus LAMP2 comprises 359 amino acids and has a calculated molecular mass of 39.8 KDa. The apparent molecular mass of it is approximately 64-68 KDa respectively in SDS-PAGE.
predicted N Leu 29
SDS-PAGE
Purity > 85 % as determined by SDS-PAGE
Protein Construction A DNA sequence encoding the cynomolgus LAMP2 (G7Q3L7) (Leu29-Phe376) was expressed with a polyhistidine tag at the C-terminus.
Bio-activity
Research Area Signaling |Signal Transduction |Metabolism |Pathways and Processes |Metabolism processes |Autophagy and mitophagy |
Formulation Lyophilized from sterile PBS, pH 7.4.
1. Normally 5 % - 8 % trehalose and mannitol are added as protectants before lyophilization. Specific concentrations are included in the hardcopy of COA.
Background LAMP2 (Lysosomal-associated membrane protein 2), also known as CD107b (Cluster of Differentiation 107b), is a member of a family of membrane glycoproteins. This glycoprotein provides selectins with carbohydrate ligands. In human, 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.
Reference
  • 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.