Abcam, ab317174, Anti-SPG7 antibody [HL2236]

Size: 100µL
Rabbit Monoclonal SPG7/PGN antibody. Suitable for ICC/IF, WB and reacts with Human, Mouse, Rat samples. Immunogen corresponding to Recombinant Fragment Protein within Human SPG7.
Key facts
Host species:Rabbit,
Clonality:Monoclonal,
Clone number:HL2236,
Isotype:IgG,
Carrier free:No,
Reacts with:Human, Mouse, Rat,
Applications:WB, ICC/IFSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Recombinant Fragment Protein within Human SPG7.Q9UQ90

Properties and Storage Information:
Form-Liquid, Storage buffer-pH: 7.4Constituents: PBS, Shipped at conditions-Blue Ice, Appropriate short-term storage duration-1-2 weeks, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
SPG7 also known as paraplegin is a mitochondrial metalloprotease. It has a molecular mass of approximately 88 kDa. SPG7 is encoded by the SPG7 gene located in humans on chromosome 16. It expresses widely in tissues with a strong presence in the nervous system and muscles. Alternate names for this protein are PGN and spastic paraplegia 7 due to its association with specific neurodegenerative conditions.
Biological function summary
SPG7 functions as a part of the mitochondrial inner membrane m-AAA protease complex. This complex performs essential roles in the maintenance of mitochondrial homeostasis by degrading damaged or misfolded proteins within the mitochondria. The presence of SPG7 is important for proper mitochondrial function. The protein has proteolytic activity which supports mitochondrial respiratory chain complexes' assembly and stability.
Pathways
SPG7 directly involves itself in mitochondrial protein quality control and the broader regulation of mitochondrial function. It interacts closely with ATP-dependent proteases like AFG3L2 playing a significant part in mitochondrial biogenesis pathways. These partnerships are key for maintaining cellular energy balance and ensuring efficient electron transport chain operation.
SPG7 mutations connect predominantly to hereditary spastic paraplegia (HSP) and have also been linked to ataxia. These mutations interrupt normal mitochondrial function leading to the degeneration of motor pathways. In HSP SPG7 interacts with proteins such as paraplegin-associated proteins SPG5 and SPG11 contributing to the disorder's pathogenesis. Understanding SPG7’s role in these diseases aids in exploring therapeutic strategies for mitochondrial-related disorders.