How a target protein is delivered into lysosome during chaperone-mediated autophagy?
A chaperone protein binds first to its cytosolic target substrate, followed by a receptor on the lysosomal membrane at the site of protein unfolding. This protein is subsequently translocated into the lysosome for its degradation.
What is Kferq sequence?
The KFERQ sequence of the substrate protein is protected by its three-dimensional structure, preventing its interaction with the CMA heat shock protein complex (HSPC) (a). The CMA-specific protein is unravelled at the lysosomal membrane by HSPC and transported into the lysosome via a channel formed from LAMP2A.
What is the disease CMA?
Chromosomal microarray (CMA), which detects clinically significant copy-number variants (CNVs), has been recognized as a first-tier test for any individual with developmental delay (DD), autism spectrum disorder (ASD), or multiple birth defects (MCA; PMID: 20466091).
What is a CMA genetic test?
Chromosomal microarray analysis (CMA) provides comprehensive genetic testing for the most common chromosomal conditions as well as a large number of severe genetic conditions not detected by traditional chromosome analysis.
How does chaperone mediated autophagy work?
Chaperone-mediated autophagy (CMA) refers to the chaperone-dependent selection of soluble cytosolic proteins that are then targeted to lysosomes and directly translocated across the lysosome membrane for degradation.
Can you please explain briefly the difference of microautophagy Macroautophagy and mega autophagy?
Unlike both microautophagy and macroautophagy that recycle macromolecular constituents back to the cytosol from the vacuole, mega-autophagy is an extreme form of massive degradation resulting in cell death. In contrast, the selective autophagy pathways rely on a variety of selective autophagy receptors.
Which is true of chaperone-mediated autophagy ( CMA )?
Chaperone-mediated autophagy. Chaperone-mediated autophagy (CMA) refers to the chaperone-dependent selection of soluble cytosolic proteins that are then targeted to lysosomes and directly translocated across the lysosome membrane for degradation.
What happens to carbohydrates in CMA deficient mice?
Proteomics analyses identified several enzymes of the carbohydrate and the lipid metabolism pathways to be CMA substrates, and their altered degradation in the knockout mice explaining the abnormal metabolic phenotype of the CMA-deficient mice.
When do cells switch to selective form of autophagy?
If the starvation state persists for more than 10 hours, the cells switch to the selective form of autophagy, namely CMA, which is known to reach a plateau of maximal activation ~36 hours into fasting and remains at these levels until ~3 days.