Testing Data
Rabbit AFG3L2 Polyclonal Antibody | anti-AFG3L2 antibody
AFG3L2 Antibody
IHC: 1:20-1:200
IF: 1:20-1:50
Testing Data
Testing Data
NCBI and Uniprot Product Information
NCBI Description
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. [provided by RefSeq, Jul 2008]
Uniprot Description
AFG3L2: ATP-dependent protease which is essential for axonal development. Defects in AFG3L2 are the cause of spinocerebellar ataxia type 28 (SCA28). It is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA28 is an autosomal dominant cerebellar ataxia (ADCA) with a slow progressive course and no evidence of sensory involvement or cognitive impairment. Defects in AFG3L2 are the cause of spastic ataxia autosomal recessive type 5 (SPAX5). A neurodegenerative disorder characterized by early onset spasticity, peripheral neuropathy, ptosis, oculomotor apraxia, dystonia, cerebellar atrophy, and progressive myoclonic epilepsy.
Protein type: Membrane protein, integral; Protease; Mitochondrial; Cell development/differentiation; Chaperone; Membrane protein, multi-pass; EC 3.4.24.-
Chromosomal Location of Human Ortholog: 18p11
Cellular Component: mitochondrion; mitochondrial inner membrane; integral to membrane
Molecular Function: protein binding; zinc ion binding; metalloendopeptidase activity; unfolded protein binding; ATP binding
Biological Process: myelination; axonogenesis; cristae formation; mitochondrial fusion; righting reflex; death; regulation of multicellular organism growth; nerve development; proteolysis; muscle fiber development; neuromuscular junction development
Disease: Spinocerebellar Ataxia 28; Spastic Ataxia 5, Autosomal Recessive
Research Articles on AFG3L2
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Product Notes
The AFG3L2 afg3l2 (Catalog #AAA2530234) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The AFG3L2 Antibody reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's AFG3L2 can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF). WB: 1:500-1:5000 IHC: 1:20-1:200 IF: 1:20-1:50. Researchers should empirically determine the suitability of the AFG3L2 afg3l2 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "AFG3L2, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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