5M9A

Human angiogenin PD variant H13R

5M9A の概要

• エントリーDOI: 10.2210/pdb5m9a/pdb
• 分子名称: Angiogenin, D(-)-TARTARIC ACID (3 entities in total)
• 機能のキーワード: parkinson's, rnase, angiogenesis, hydrolase
• 由来する生物種: Homo sapiens (Human)
• 細胞内の位置: Cytoplasmic vesicle, secretory vesicle lumen : P03950
• タンパク質・核酸の鎖数: 1
• 化学式量合計: 14469.37

構造登録者

Bradshaw, W.J.,Rehman, S.,Pham, T.T.K.,Thiyagarajan, N.,Lee, R.L.,Subramanian, V.,Acharya, K.R. (登録日: 2016-11-01, 公開日: 2017-02-22, 最終更新日: 2024-10-23)

主引用文献

Bradshaw, W.J.,Rehman, S.,Pham, T.T.,Thiyagarajan, N.,Lee, R.L.,Subramanian, V.,Acharya, K.R.
Structural insights into human angiogenin variants implicated in Parkinson's disease and Amyotrophic Lateral Sclerosis.
Sci Rep, 7:41996-41996, 2017

PubMed Abstract: Mutations in Angiogenin (ANG), a member of the Ribonuclease A superfamily (also known as RNase 5) are known to be associated with Amyotrophic Lateral Sclerosis (ALS, motor neurone disease) (sporadic and familial) and Parkinson's Disease (PD). In our previous studies we have shown that ANG is expressed in neurons during neuro-ectodermal differentiation, and that it has both neurotrophic and neuroprotective functions. In addition, in an extensive study on selective ANG-ALS variants we correlated the structural changes to the effects on neuronal survival and the ability to induce stress granules in neuronal cell lines. Furthermore, we have established that ANG-ALS variants which affect the structure of the catalytic site and either decrease or increase the RNase activity affect neuronal survival. Neuronal cell lines expressing the ANG-ALS variants also lack the ability to form stress granules. Here, we report a detailed experimental structural study on eleven new ANG-PD/ALS variants which will have implications in understanding the molecular basis underlying their role in PD and ALS.

PubMed: 28176817
DOI: 10.1038/srep41996

実験手法

X-RAY DIFFRACTION (1.95 Å)