Target：SPG7

Gene Name：SPG7 CAR CMAR PGN

Protein Name：Paraplegin (EC 3.4.24.-) (Spastic paraplegia 7 protein)

Human Gene Id：6687

Human Swiss Prot No：Q9UQ90

Mouse Swiss Prot No：Q3ULF4

Rat Swiss Prot No：Q7TT47

Immunogen：Synthesized peptide derived from human protein . at AA range: 71-120

Specificity：SPG7 Polyclonal Antibody detects endogenous levels of protein.

Formulation：Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.

Source：Polyclonal, Rabbit,IgG

Dilution：WB 1:500-2000 ELISA 1:5000-20000

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Observed Band(KD)：87kD

Background： 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],

Function：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 AT

Subcellular Location：Mitochondrion inner membrane ; Multi-pass membrane protein .

Expression：Ubiquitous.