|Other Names||AFG3-like protein 2, 3424-, Paraplegin-like protein, AFG3L2|
|Target/Specificity||The synthetic peptide sequence used to generate the antibody AP13219a was selected from the N-term region of AFG3L2. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. 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 andclosely related to paraplegin. The paraplegin gene is responsiblefor an autosomal recessive form of hereditary spastic paraplegia.This gene is a candidate gene for other hereditary spasticparaplegias 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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