Table of Contents
Definition / general | Uses by pathologists | Microscopic (histologic) images | Positive staining - normal | Negative staining | Additional referencesCite this page: Pernick N. CD107b. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cdmarkerscd107b.html. Accessed January 22nd, 2021.
Definition / general
- Also called lysosome associated membrane protein 2 (LAMP-2)
- X linked
- Major constituent of lysosomal membranes (also LAMP-1)
- Functions:
- Mutations cause Danon disease, a lysosomal glycogen storage disease with cardiomyopathy in adult women and men before age 20 years (N Engl J Med 2005;352:362, Circulation 2005;112:1612), Wolff-Parkinson-White syndrome, myopathy, variable intellectual disability and retinopathy (Arch Ophthalmol 2007;125:231)
- Skeletal and cardiac muscle cells have intracytoplasmic vacuoles containing glycogen and autophagic material
- Levels decrease with age (J Cell Sci 2007;120:782)
- Binding of substrates to LAMP-2 is limiting step in chaperone-mediated autophagy, a selective mechanism for the degradation of soluble cytosolic proteins in lysosomes (EMBO J 2006;25:3921)
- May be involved in infection induced involution of thymus via autophagy (Acta Biol Hung 2006;57:315)
- Facilitates MHC class II presentation of cytoplasmic antigens (Immunity 2005;22:571)
- Basophil activation antigen (Cytometry A 2004;61:62)
- Mutations cause Danon disease, a lysosomal glycogen storage disease with cardiomyopathy in adult women and men before age 20 years (N Engl J Med 2005;352:362, Circulation 2005;112:1612), Wolff-Parkinson-White syndrome, myopathy, variable intellectual disability and retinopathy (Arch Ophthalmol 2007;125:231)
Uses by pathologists
- Help differentiate cardiomyopathy due to Danon disease (LAMP-2 negative) from other causes (LAMP-2+, Intern Med 2007;46:757)
Positive staining - normal
- Lysosome membranes of various tissues, platelets (Thromb Haemost 1996;75:623)
Negative staining
- Cardiac and skeletal muscle in Danon disease (Neuromuscul Disord 2003;13:708)
Additional references