3VFD

Human spastin AAA domain

3VFD の概要

• エントリーDOI: 10.2210/pdb3vfd/pdb
• 分子名称: Spastin, SULFATE ION (2 entities in total)
• 機能のキーワード: atpase, microtubule severing, hydrolase
• 由来する生物種: Homo sapiens (human)
• 細胞内の位置: Membrane ; Peripheral membrane protein . Isoform 1: Endoplasmic reticulum membrane ; Peripheral membrane protein . Isoform 3: Cytoplasm : Q9UBP0
• タンパク質・核酸の鎖数: 1
• 化学式量合計: 42670.72

構造登録者

Taylor, J.L.,White, S.R.,Lauring, B.,Kull, F.J. (登録日: 2012-01-09, 公開日: 2012-05-30, 最終更新日: 2023-09-13)

主引用文献

Taylor, J.L.,White, S.R.,Lauring, B.,Kull, F.J.
Crystal structure of the human spastin AAA domain.
J.Struct.Biol., 179:133-137, 2012

PubMed Abstract: Hereditary spastic paraplegia (HSP) is a motor neuron disease caused by a progressive degeneration of the motor axons of the corticospinal tract. Point mutations or exon deletions in the microtubule-severing ATPase, spastin, are responsible for approximately 40% of cases of autosomal dominant HSP. Here, we report the 3.3 Å X-ray crystal structure of a hydrolysis-deficient mutant (E442Q) of the human spastin protein AAA domain. This structure is analyzed in the context of the existing Drosophila melanogaster spastin AAA domain structure and crystal structures of other closely related proteins in order to build a more unifying framework for understanding the structural features of this group of microtubule-severing ATPases.

PubMed: 22446388
DOI: 10.1016/j.jsb.2012.03.002

実験手法

X-RAY DIFFRACTION (3.301 Å)