产品名称
SPG7 Rabbit Polyclonal Antibody
蛋白名称
Paraplegin (EC 3.4.24.-) (Spastic paraplegia 7 protein)
存储缓冲液
Liquid in PBS containing 50% glycerol, and 0.02% New type preservative N.
Human Swissprot No.
Q9UQ90
Human Swissprot Link
https://www.uniprot.org/uniprotkb/Q9UQ90/entry
Mouse Swissprot No.
Q3ULF4
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q3ULF4
Rat Swissprot Link
http://www.uniprot.org/uniprot/O54941Q7TT47
免疫原
Synthesized peptide derived from human protein . at AA range: 71-120
特异性
SPG7 Polyclonal Antibody detects endogenous levels of protein.
稀释度
WB 1:500-2000 ELISA 1:5000-20000
宿主
Polyclonal, Rabbit,IgG
背景介绍
This gene encodes a mitochondrial metalloprotease protein that is a member of the AAA family. Members of this protein family share an ATPase domain and have roles in diverse cellular processes including membrane trafficking, intracellular motility, organelle biogenesis, protein folding, and proteolysis. Mutations in this gene cause autosomal recessive spastic paraplegia 7. Two transcript variants encoding distinct isoforms have been identified. [provided by RefSeq, Mar 2014],
细胞定位
Mitochondrion inner membrane ; Multi-pass membrane protein .
功能
caution:A CDS in the 3'-UTR of SPG7 mRNA had been erroneously identified as a cell matrix adhesion regulator and originally thought to be encoded by the CMAR gene. There is no experimental evidence for the production of endogenous CMAR protein.,disease:Defects in SPG7 are the cause of spastic paraplegia autosomal recessive type 7 (SPG7) [MIM:607259]. Spastic paraplegia is a degenerative spinal cord disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. SPG7 is a complex form. Additional clinical features are cerebellar syndrome, supranuclear palsy, and cognitive impairment, particularly disturbance of attention and executive functions.,function:Putative ATP-dependent protease.,sequence caution:Translated as Glu.,similarity:In the C-terminal section; belongs to the peptidase M41 family.,similarity:In the N-terminal section; belongs to the AAA ATPase family.,tissue specificity:Ubiquitous.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
