SPG7, Polyclonal Antibody

Polyclonal SPG7; Anti-SPG7; MGC126332; SPG5C; SPG-7; cell adhesion regulator; SPG 7; paraplegin; MGC126331; PGN; SPG7; CAR; CMAR; FLJ37308

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

SPG7 antibody was purified by antigen-affinity chromatography

Supplied as a concentrated soloution containing 0.1M Tris, 0.1M Glycine, 20% Glycerol (pH 7.0). 0.01% Thimerosal was added as a preservative.

0.83 mg/ml (lot specific)

Store at 4 degree C for short term storage. Aliquot and store at -20 degree C for long term storage. Avoid repeated freeze/thaw cycles.

Small volumes of anti-SPG7 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Affinity purified Rabbit polyclonal SPG7 antibody

Proteases, Inhibitors, & Enzymes; Purified Polyclonal Antibodies

Western Blot (WB)

WB: 1:500-1:3000

Western blot analysis of 50 ug of whole cell lysate (A: Mouse brain) using a 7.5 % SDS PAGE gel and SPG7 antibody at a dilution of 1:1000

Western blot analysis of 30 ug of whole cell lysate (A: 293T) using a 7.5 % SDS PAGE gel and SPG7 antibody at a dilution of 1:1000

88 kDa

spastic paraplegia 7 (pure and complicated autosomal recessive)

paraplegin

Paraplegin

CAR; CMAR; PGN  [Similar Products]

SPG7_HUMAN

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]

SPG7: Putative ATP-dependent zinc metalloprotease. Defects in SPG7 are the cause of spastic paraplegia autosomal recessive type 7 (SPG7). 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. Defects in SPG7 may cause autosomal recessive osteogenesis imperfecta (OI). Osteogenesis imperfecta defines a group of connective tissue disorders characterized by bone fragility and low bone mass. Clinical features of SPG7-related osteogenesis imperfecta include recurrent fractures, mild bone deformities, delayed tooth eruption, normal hearing and white sclera. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, integral; EC 3.4.24.-; Membrane protein, multi-pass; Cell adhesion; Mitochondrial; Protease; Motility/polarity/chemotaxis; Chaperone

Chromosomal Location of Human Ortholog: 16q24.3

Cellular Component: mitochondrion; mitochondrial membrane; integral to membrane

Molecular Function: peptidase activity; protein binding; zinc ion binding; metalloendopeptidase activity; unfolded protein binding; ATP binding

Biological Process: mitochondrion organization and biogenesis; nervous system development; proteolysis; anterograde axon cargo transport

Disease: Spastic Paraplegia 7, Autosomal Recessive

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