Anti-SPG7 Polyclonal Antibody for WB, IHC-P, IF/ICC, ELISA - Q9UQ90

Core Product Specifications and Parameters

Biological Background: SPG7/Paraplegin Function and Localization

• SPG7 encodes paraplegin (also known as CAR, PGN, CMAR), a catalytic component of the mitochondrial inner membrane m-AAA protease, which is an ATP-dependent zinc metalloprotease.
• Paraplegin possesses both ATPase and protease activities and mediates proteostasis of inner mitochondrial membrane proteins, essential for axonal and neuron development. Related references: PMID:11549317, PMID:28396416, PMID:31097542
• It catalyzes the maturation of mitochondrial ribosomal protein MRPL32 by cleaving its presequence, facilitating ribosome assembly (By similarity).
• SPG7 regulates mitochondrial calcium homeostasis by mediating degradation of SMDT1/EMRE and MCU, affecting the assembly and activity of the calcium uniporter. Related references: PMID:28396416, PMID:31097542
• It participates in the formation and regulation of the mitochondrial permeability transition pore (mPTP), with this function independent of its proteolytic activity. Related references: PMID:26387735
• Localized to the mitochondrial inner membrane (UniProt Q9UQ90).
• Ubiquitously expressed across tissues (UniProt Q9UQ90).
• Key keywords include alternative splicing, ATP-binding, disease variant (hereditary spastic paraplegia, osteogenesis imperfecta), metal-binding, metalloprotease, nitration, and transit peptide.

Experimental Guidance and Technical Tips

• The immunogen corresponds to the N-terminal region (aa 1-250) of human SPG7, which includes the transit peptide; the antibody may recognize both precursor and mature forms.
• For Western blot, a band around 88 kDa is expected; cross-reactivity with human, mouse, and rat has been indicated. Validate in your specific sample system and include appropriate controls.
• For IHC-P and IF/ICC, consider paraffin-embedded or frozen sections and optimize antigen retrieval conditions.
• For ELISA, the recombinant protein immunogen can be used as a positive control to assess antibody specificity and titer.

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