tRNA Dysregulation in Neurodevelopmental and Neurodegenerative Diseases.

IF 11.4 1区 生物学 Q1 CELL BIOLOGY
Robert W Burgess, Erik Storkebaum
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引用次数: 3

Abstract

Transfer RNAs (tRNAs) decode messenger RNA codons to peptides at the ribosome. The nuclear genome contains many tRNA genes for each amino acid and even each anticodon. Recent evidence indicates that expression of these tRNAs in neurons is regulated, and they are not functionally redundant. When specific tRNA genes are nonfunctional, this results in an imbalance between codon demand and tRNA availability. Furthermore, tRNAs are spliced, processed, and posttranscriptionally modified. Defects in these processes lead to neurological disorders. Finally, mutations in the aminoacyl tRNA synthetases (aaRSs) also lead to disease. Recessive mutations in several aaRSs cause syndromic disorders, while dominant mutations in a subset of aaRSs lead to peripheral neuropathy, again due to an imbalance between tRNA supply and codon demand. While it is clear that disrupting tRNA biology often leads to neurological disease, additional research is needed to understand the sensitivity of neurons to these changes.

神经发育和神经退行性疾病中的tRNA失调。
转移核糖核酸(tRNA)将信使核糖核酸密码子解码为核糖体上的肽。核基因组包含每个氨基酸甚至每个反密码子的许多tRNA基因。最近的证据表明,这些tRNA在神经元中的表达受到调节,并且它们在功能上不是多余的。当特定的tRNA基因不起作用时,这会导致密码子需求和tRNA可用性之间的失衡。此外,tRNA被剪接、加工和转录后修饰。这些过程中的缺陷会导致神经系统紊乱。最后,氨酰基tRNA合成酶(aaRS)的突变也会导致疾病。几种aaRS的隐性突变会导致综合征疾病,而一部分aaRS的显性突变会导致周围神经病变,这也是由于tRNA供应和密码子需求之间的不平衡。虽然很明显,破坏tRNA生物学通常会导致神经疾病,但还需要更多的研究来了解神经元对这些变化的敏感性。
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来源期刊
CiteScore
19.50
自引率
0.00%
发文量
21
期刊介绍: The Annual Review of Cell and Developmental Biology, established in 1985, comprehensively addresses major advancements in cell and developmental biology. Encompassing the structure, function, and organization of cells, as well as the development and evolution of cells in relation to both single and multicellular organisms, the journal explores models and tools of molecular biology. As of the current volume, the journal has transitioned from gated to open access through Annual Reviews' Subscribe to Open program, making all articles published under a CC BY license.
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