AFG3L2, Polyclonal Antibody

Anti -AFG3L2 (AFG3-like Protein 2, Paraplegin-like Protein, FLJ25993)

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

Chromosome: 18; NC_000018.9 (12328943..12377275, complement). Location: 18p11

Polyclonal

IgG

Mouse

Recognizes human AFG3L2.

Affinity Purified
Purified by Protein A affinity chromatography.

Supplied as a liquid in PBS, pH 7.2.

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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.

ATP-dependent protease which is essential for axonal development.

Antibodies; Abs to Enzymes, ATPase

Western Blot (WB)

Suitable for use in Western Blot.

88,584 Da[Similar Products]

AFG3 ATPase family member 3-like 2 (S. cerevisiae)

AFG3-like protein 2; paraplegin-like protein; ATPase family gene 3, yeast; AFG3 ATPase family gene 3-like 2

AFG3-like protein 2

AFG32_HUMAN

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

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; cristae formation; mitochondrial fusion; axonogenesis; 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

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