Genes of endoplasmic reticulam shaping proteins are among the most commonly mutated in hearediatry spastic paraplegia.The process of protein synthesis directly drives the transfer of growing polypeptide chains through the sec61 channel into the ER.
These folding factors which consist of classical chaperones and thier cochaperones , the carbohydrate -binding cochapersons and the folding catalysts of PDI and proline cis-trans isomerase families.
Protein that fail to fold correctly are trans located back across the endoplasmic reticulum membrane to the cytosol , they become sub strates for the cytosolic degradative machinery.Misfolded secretly proteins remain the endoplasmic reticulum and degraded by ER- associated . They targeted for destruction by this machine which chews protein ans splits them out as small fragments of amino acids.
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