Full Product Name
SPG7 antibody
Product Synonym Names
Monoclonal SPG7; Anti-SPG7; CAR; CMAR; FLJ37308; MGC126331; MGC126332; PGN; SPG5C; SPG7; SPG 7; SPG-7
Product Gene Name
anti-SPG7 antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for Q9UQ90
Species Reactivity
Human, Mouse
Purity/Purification
SPG7 antibody was purified by affinity chromatography.
Form/Format
Supplied in PBS buffer, pH 7.3, containing 1% BSA, 50% glycerol and 0.02% sodium azide.
Concentration
0.5-1 mg/ml (lot specific)
Biological Significance
This gene encodes a nuclear-encoded mitochondrial metalloprotease protein that is a member of the AAA (ATPases associated with a variety of cellular activities) protein 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. Two transcript variants encoding distinct isoforms have been identified for this gene. Mutations associated with this gene cause autosomal recessive spastic paraplegia 7.
Immunogen
SPG7 antibody was raised in Mouse using Human recombinant protein fragment corresponding to amino acids 300-573 of human SPG7 prodduced in E Coli as the immunogen.
Preparation and Storage
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.
Other Notes
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.
Related Product Information for
anti-SPG7 antibody
Mouse monoclonal SPG7 antibody
Product Categories/Family for anti-SPG7 antibody
Proteases, Inhibitors, & Enzymes
Applications Tested/Suitable for anti-SPG7 antibody
Flow Cytometry (FC/FACS), Immunofluorescence (IF), Western Blot (WB)
Application Notes for anti-SPG7 antibody
FC: 1:100
IF: 1:50-100
WB: 1:500-2000
Flow Cytometry (FC/FACS) of anti-SPG7 antibody
Flow Cytometric analysis of HEK293T cells transfected with either recombinant SPG7 protein (red) or empty vector (blue) stained using SPG7 antibody
Western Blot (WB) of anti-SPG7 antibody
Western Blot analysis of HEK293T cell lysates (5 ug) transfected with either recombinant SPG7 protein (Right) or empty vector (Left) detected with SPG7 antibody
Immunofluorescence (IF) of anti-SPG7 antibody
Immunofluorescent staining of COS7 cells transiently transfected with recombinant SPG7 protein using SPG7 antibody
NCBI/Uniprot data below describe general gene information for SPG7. It may not necessarily be applicable to this product.
NCBI Accession #
AAH35929.1
[Other Products]
UniProt Primary Accession #
Q9UQ90
[Other Products]
UniProt Secondary Accession #
O75756; Q2TB70; Q58F00; Q96IB0[Other Products]
UniProt Related Accession #
Q9UQ90[Other Products]
Molecular Weight
53,940 Da
NCBI Official Full Name
SPG7 protein, partial
NCBI Official Synonym Full Names
spastic paraplegia 7 (pure and complicated autosomal recessive)
NCBI Official Symbol
SPG7 [Similar Products]
NCBI Official Synonym Symbols
CAR; PGN; CMAR; SPG5C
[Similar Products]
NCBI Protein Information
paraplegin
UniProt Protein Name
Paraplegin
UniProt Synonym Protein Names
Spastic paraplegia 7 protein
Protein Family
Paraplegin
UniProt Gene Name
SPG7 [Similar Products]
UniProt Synonym Gene Names
CAR; CMAR; PGN [Similar Products]
UniProt Entry Name
SPG7_HUMAN
NCBI Summary for SPG7
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]
UniProt Comments for SPG7
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, multi-pass; Protease; Mitochondrial; Chaperone; Membrane protein, integral; Cell adhesion; Motility/polarity/chemotaxis; EC 3.4.24.-
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: nervous system development; mitochondrion organization and biogenesis; proteolysis; anterograde axon cargo transport
Disease: Spastic Paraplegia 7, Autosomal Recessive
Research Articles on SPG7
1. In unexplained ataxia, there was a significant number of patients with SPG7 mutations.
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
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