Rabbit AFG3L2 Polyclonal Antibody | anti-AFG3L2 antibody

Anti-AFG3L2 Antibody

Cat. Num. AAA178415

• Gene Names: AFG3L2; SCA28; SPAX5
• Reactivity: Human, Mouse, Rat; No cross reactivity with other proteins.
• Applications: Western Blot
• Purity: Immunogen affinity purified.
• Synonyms: AFG3L2; Polyclonal Antibody; Anti-AFG3L2 Antibody; AFG3 like protein 2; AFG3-like protein 2; SCA28; Spinocerebellar ataxia 28; Q9Y4W6; AFG3 like matrix AAA peptidase subunit 2; anti-AFG3L2 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat; No cross reactivity with other proteins.
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Immunogen affinity purified.
• Form/Format: Lyophilized
• Sequence Length: 797

• Applicable Applications for anti-AFG3L2 antibody: Western Blot (WB)
• Application Notes: Western Blot: 0.1-0.5mug/ml; Tested Species: Human, Mouse, Rat; Tested Species:In-house tested species with positive results.; Other applications have not been tested.
• Immunogen: E Coli-derived human AFG3L2 recombinant protein (Position: R168-D250). Human AFG3L2 shares 100% amino acid (aa) sequence identity with mouse AFG3L2.
• Contents: Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.
• Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
• Preparation and Storage: At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time. Avoid repeated freezing and thawing.

Western Blot (WB)

(Western blot analysis of AFG3L2 expression in rat brain extract (lane 1), mouse spleen extract (lane 2) and U87 whole cell lysates (lane 3). AFG3L2 at 88KD was detected using rabbit anti- AFG3L2 Antigen Affinity purified polyclonal antibody at 0.5 ug/mL. The blot was developed using chemiluminescence (ECL) method. )

Related Product Information for anti-AFG3L2 antibody

Rabbit IgG polyclonal antibody for AFG3-like protein 2 (AFG3L2) detection.
Background: AFG3L2 is the catalytic subunit of the m-AAA protease, an ATP-dependent proteolytic complex of the mitochondrial inner membrane that degrades misfolded proteins and regulates ribosome assembly. In humans, it is encoded by the AFG3L2 gene. 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. And this gene is a candidate gene for other hereditary spastic paraplegias or neurodegenerative disorders as well as spastic ataxia-neuropathy syndrome.

References

1. "Entrez Gene: AFG3 ATPase family gene 3-like 2 (S. cerevisiae)".
2. Koppen, M., Metodiev, M. D., Casari, G., Rugarli, E. I., Langer, T. Variable and tissue-specific subunit composition of mitochondrial m-AAA protease complexes linked to hereditary spastic paraplegia. Molec. Cell. Biol. 27: 758-767, 2007.
3. Pierson TM, Adams D, Bonn F, Martinelli P, Cherukuri PF, Teer JK, Hansen NF, Cruz P, Mullikin For The Nisc Comparative Sequencing Program JC, Blakesley RW, Golas G, Kwan J, Sandler A, Fuentes Fajardo K, Markello T, Tifft C, Blackstone C, Rugarli EI, Langer T, Gahl WA, Toro C (October 2011). "Whole-exome sequencing identifies homozygous AFG3L2 mutations in a spastic ataxia-neuropathy syndrome linked to mitochondrial m-AAA proteases". PLoS Genet. 7 (10): e1002325.

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

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

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: cristae formation; mitochondrial fusion; nerve development; protein complex assembly; protein import into mitochondrial intermembrane space

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