Rabbit anti-Human AFG3L2 Polyclonal Antibody | anti-AFG3L2 antibody

AFG3L2 Polyclonal Antibody

Cat. Num. AAA9419900

• Gene Names: AFG3L2; SCA28; SPAX5
• Reactivity: Human
• Applications: Western Blot
• Purity: Affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
• Synonyms: AFG3L2; Polyclonal Antibody; AFG3L2 Polyclonal Antibody; AFG3-like protein 2 (EC 3.4.24.-) (Paraplegin-like protein); anti-AFG3L2 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Specificity: AFG3L2 Polyclonal Antibody detects endogenous levels of AFG3L2
• Purity/Purification: Affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
• Form/Format: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
• Concentration: 1 mg/ml (varies by lot)

• Applicable Applications for anti-AFG3L2 antibody: Western Blot (WB)
• Application Notes: WB: 1:500-1:2000
• Immunogen: Synthesized peptide derived from human AFG3L2. at AA range: 744-793
• Immunogen Type: Protein
• Preparation and Storage: Store at -20 degree C

Western Blot (WB)

(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))

Product Categories/Family for anti-AFG3L2 antibody

Total protein Ab

NCBI and Uniprot Product Information

• NCBI GI #: 300192933
• NCBI GeneID: 10939
• NCBI Accession #: NP_006787.2
• NCBI GenBank Nucleotide #: NM_006796.2
• UniProt Accession #: Q9Y4W6; Q6P1L0
• Molecular Weight: 88 kDa
• NCBI Official Full Name: AFG3-like protein 2
• NCBI Official Synonym Full Names: AFG3 like matrix AAA peptidase subunit 2
• NCBI Official Symbol: AFG3L2
• NCBI Official Synonym Symbols: SCA28; SPAX5
• NCBI Protein Information: AFG3-like protein 2
• UniProt Protein Name: AFG3-like protein 2
• Protein Family: AFG3-like protein
• UniProt Gene Name: AFG3L2

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: 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