Western Blot (WB)
(SPG7 Antibody (Center) western blot analysis in 293 cell line lysates (35ug/lane).This demonstrates the SPG7 antibody detected the SPG7 protein (arrow).)
Rabbit anti-Human SPG7 Polyclonal Antibody | anti-SPG7 antibody
SPG7 Antibody (Center)
Western Blot (WB)
(SPG7 Antibody (Center) western blot analysis in 293 cell line lysates (35ug/lane).This demonstrates the SPG7 antibody detected the SPG7 protein (arrow).)
Western Blot (WB)
(SPG7 Antibody (Center) western blot analysis in 293 cell line lysates (35ug/lane).This demonstrates the SPG7 antibody detected the SPG7 protein (arrow).)
Immunohistochemistry (IHC)
(SPG7 Antibody (Center) immunohistochemistry analysis in formalin fixed and paraffin embedded human brain tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of SPG7 Antibody (Center) for immunohistochemistry. Clinical relevance has not been evaluated.)
Immunohistochemistry (IHC)
(SPG7 Antibody (Center) immunohistochemistry analysis in formalin fixed and paraffin embedded human brain tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of SPG7 Antibody (Center) for immunohistochemistry. Clinical relevance has not been evaluated.)
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. [provided by
RefSeq].
Augustin, S., et al. Mol. Cell 35(5):574-585(2009)
Karlberg, T., et al. PLoS ONE 4 (10), E6975 (2009) :
Brugman, F., et al. Neurology 71(19):1500-1505(2008)
Tzoulis, C., et al. J. Neurol. 255(8):1142-1144(2008)
NCBI and Uniprot Product Information
NCBI Description
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 Description
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: Motility/polarity/chemotaxis; Membrane protein, integral; EC 3.4.24.-; Chaperone; Protease; Mitochondrial; Cell adhesion; Membrane protein, multi-pass
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
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Product Notes
The SPG7 spg7 (Catalog #AAA9209838) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 115-141. The SPG7 Antibody (Center) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's SPG7 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC). WB~~1:1000. Researchers should empirically determine the suitability of the SPG7 spg7 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "SPG7, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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