Rabbit anti-Human AFG3-like protein 2 Polyclonal Antibody | anti-AFG3L2 antibody

AFG3-like protein 2 Polyclonal Antibody

Cat. Num. AAA9416118

• Gene Names: AFG3L2; SCA28; SPAX5
• Reactivity: Human
• Applications: Western Blot, Immunohistochemistry
• Purity: Caprylic Acid Ammonium Sulfate Precipitation purified
• Synonyms: AFG3-like protein 2; Polyclonal Antibody; AFG3-like protein 2 Polyclonal Antibody; Paraplegin-like protein; AFG3L2; anti-AFG3L2 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Specificity: The antibody detects endogenous level of total AFG3-like protein 2 polyclonal antibody.
• Purity/Purification: Caprylic Acid Ammonium Sulfate Precipitation purified
• Form/Format: Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
• Concentration: 1.0 mg/ml (varies by lot)
• Sequence Length: 797

• Applicable Applications for anti-AFG3L2 antibody: Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: Western Blot: 1:500 - 1:1000; Immunohistochemistry: 1:20 - 1:200
• Immunogen: Recombinant Human AFG3-like protein 2 protein
• Immunogen Type: Protein
• Preparation and Storage: Store at -20 degree C

Western Blot (WB)

(AII lanes: AFG3-like protein 2 Antibody at 2ug/ml + EC109 whole cell lysate at 20 ug)

Immunohistochemistry (IHC)

(Immunohistochemical analysis of paraffin-embedded humanprostate using at dilution of 1:100.)

Related Product Information for anti-AFG3L2 antibody

AFG3L2 is a protein localized in mitochondria and closely related to paraplegin. The paraplegin gene is responsible for an autosomal recessive form of hereditary spastic paraplegia. AFG3L2 is a candidate gene for other hereditary spastic paraplegias or neurodegenerative disorders and is a putative ATP dependent protease.

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: 88,584 Da
• 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
• UniProt Entry Name: AFG32_HUMAN

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

Cellular Component: mitochondrial inner membrane; mitochondrion

Molecular Function: ATP-dependent peptidase activity; metallopeptidase activity; protein binding; unfolded protein binding

Biological Process: axonogenesis; cristae formation; mitochondrial calcium ion homeostasis; mitochondrial fusion; nerve development; protein complex assembly; protein import into mitochondrial intermembrane space; proteolysis

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