Rabbit anti-Human SPG7 Polyclonal Antibody | anti-SPG7 antibody

SPG7 antibody

Cat. Num. AAA9410688

• Gene Names: SPG7; CAR; PGN; CMAR; SPG5C
• Reactivity: Human
• Applications: Western Blot
• Purity: Purified by antigen-affinity chromatography.
• Synonyms: SPG7; Polyclonal Antibody; SPG7 antibody; anti-SPG7 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Purity/Purification: Purified by antigen-affinity chromatography.
• Form/Format: Supplied in 0.1M Tris-buffered saline with 20% Glycerol (pH7.0). 0.01% Thimerosal was added as a preservative.
• Sequence Length: 795

• Applicable Applications for anti-SPG7 antibody: Western Blot (WB)
• Application Notes: Western blotting: 1:500-1:3000
• Immunogen Type: Recombinant protein
• Immunogen Description: Recombinant protein fragment contain a sequence corresponding to a region within amino acids 384 and 568 of SPG7
• Target Name: SPG7
• Preparation and Storage: Store at -20 degree C for long term preservation (recommended). Store at 4 degree C for short term use.

Testing Data

(Sample (30 ug of whole cell lysate) A: 293T 7.5% SDS PAGE Primary antibody diluted at 1: 1000)

Related Product Information for anti-SPG7 antibody

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. [provided by RefSeq]

Product Categories/Family for anti-SPG7 antibody

Total protein Ab

NCBI and Uniprot Product Information

• NCBI GI #: 62020635
• NCBI GeneID: 6687
• Molecular Weight: 53,940 Da
• NCBI Official Full Name: Spastic paraplegia 7 (pure and complicated autosomal recessive)
• NCBI Official Synonym Full Names: spastic paraplegia 7 (pure and complicated autosomal recessive)
• NCBI Official Symbol: SPG7
• NCBI Official Synonym Symbols: CAR; PGN; CMAR; SPG5C
• NCBI Protein Information: paraplegin
• UniProt Protein Name: Paraplegin
• Protein Family: Paraplegin
• UniProt Gene Name: SPG7
• UniProt Synonym Gene Names: CAR; CMAR; PGN
• UniProt Entry Name: SPG7_HUMAN

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

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