AFG3L2, Polyclonal Antibody

AFG3-like protein 2 (EC 3.4.24.-) (Paraplegin-like protein)

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

AFG3L2 Polyclonal Antibody detects endogenous levels of AFG3L2

Affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

1 mg/ml (lot specific)

Store at -20 degree C

Small volumes of anti-AFG3L2 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Total protein Ab

Western Blot (WB)

WB: 1:500-1:2000

Western Blot analysis of 1, mouse-heart 2, 293T cells using primary antibody diluted at 1:500 ( degree C overnight). Secondary antibody£ºGoat Anti-rabbit IgG IRDye 800 (diluted at 1:5000, 25¡£C, 1 hour)

88 kDa

AFG3 like matrix AAA peptidase subunit 2

AFG3-like protein 2

AFG3-like protein 2

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]

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: Cell development/differentiation; Chaperone; EC 3.4.24.-; Membrane protein, integral; Membrane protein, multi-pass; Mitochondrial; Protease

Chromosomal Location of Human Ortholog: 18p11.21

Cellular Component: mitochondrial inner membrane; mitochondrion

Molecular Function: metallopeptidase activity; protein binding; unfolded protein binding

Biological Process: axonogenesis; mitochondrial calcium ion homeostasis; mitochondrial calcium ion transport; proteolysis

Disease: Spastic Ataxia 5, Autosomal Recessive; Spinocerebellar Ataxia 28

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.