BIOL 1300 Lecture Notes - Lecture 6: Rna Recognition Motif, Neurodegeneration, Iceberg
Document Summary
The tip of the iceberg: rna-binding proteins with prion-like domains in neurodegenerative disease (king et al) Of 210 human proteins with rrms, 29 have putative prion domain, 12 are in the top 60 prion candidates in entire genome (prone to aggregate) These rna-binding prion candidates are in pathology/genetics of neurodegenerative disease hnrnpa1, hnrnpa2b1 emerged as disease proteins. Rrm1 and rrm2, followed by putatively-unfolded prion-like domain. Functional complexes (ribonucleosomes) with >20 other different hnrnp and hnrna in nucleus. These granules transport myelin basic protein (mbp, important in creating myelin) mrna, travel down oligodendrocyte. Disassembly at destination is signaled by fyn (tyrosine) kinase phosphorylation, release mrna. Mechanism is unknown, could be a liquid-liquid phase separated assembly. Inclusion body myositis (ibm): most common myopathy of aging. Associated with both inflammatory (-itis) and degenerative processes. Incidence increasing, correlated to use of statin drugs for cholesterol lowering.
