|Application ||WB, IHC-P, E|
|Other Accession||Q8JZQ2, Q2KJI7, NP_006787.2|
|Calculated MW||88584 Da|
|Antigen Region||52-80 aa|
|Other Names||AFG3-like protein 2, 3424-, Paraplegin-like protein, AFG3L2|
|Target/Specificity||This AFG3L2 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 52-80 amino acids from the N-terminal region of human AFG3L2.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.|
|Storage||Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||AFG3L2 Antibody (N-term) is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||ATP-dependent protease which is essential for axonal and neuron development. In neurons, mediates degradation of SMDT1/EMRE before its assembly with the uniporter complex, limiting the availability of SMDT1/EMRE for MCU assembly and promoting efficient assembly of gatekeeper subunits with MCU.|
|Cellular Location||Mitochondrion membrane; Multi-pass membrane protein|
|Tissue Location||Ubiquitous. Highly expressed in the cerebellar Purkinje cells.|
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Provided below are standard protocols that you may find useful for product applications.
This gene encodes a protein localized in mitochondria and closely related to paraplegin. The paraplegin gene is responsible for an autosomal recessive form of hereditary spastic paraplegia. This gene is a candidate gene for other hereditary spastic paraplegias or neurodegenerative disorders.
Edener, U., et al. Eur. J. Hum. Genet. 18(8):965-968(2010)
Di Bella, D., et al. Nat. Genet. 42(4):313-321(2010)
Augustin, S., et al. Mol. Cell 35(5):574-585(2009)
Mariotti, C., et al. Cerebellum 7(2):184-188(2008)
Cagnoli, C., et al. Brain 129 (PT 1), 235-242 (2006) :
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