AFG3L2 antibody pair

AFG3L2 Antibody Pair

Cat. Num. AAA7041840

• Gene Names: AFG3L2; SCA28; SPAX5
• Reactivity: Rat, Guinea Pig, Pig, Dog, Rabbit, Goat, Sheep
• Applications: ELISA
• Synonyms: AFG3L2; Polyclonal Antibody Pair; AFG3L2 Antibody Pair; AFG3-like protein 2; Paraplegin-like protein; AFG3L2 antibody pair

• Host: Capture: Rabbit; Detection: Rabbit
• Reactivity: Rat, Guinea Pig, Pig, Dog, Rabbit, Goat, Sheep
• Clonality: Polyclonal
• Isotype: Capture: IgG; Detection: IgG
• Form/Format: Liquid
• Concentration: The concentrations and volumes of the antibodies may vary and be lot specific. Details will be on the vial labels. (varies by lot)
• Sequence Length: 797

• Applicable Applications for AFG3L2 antibody pair: Sandwich ELISA (EIA)
• Application Notes: We recommend using the capture antibody at a concentration of 0.2ug/ml and the detection antibody at a concentration of 0.2ug/ml.
• Immunogen Species: Human
• Conjugation: Capture: Non-conjugated; Detection: Biotin conjugated
• Recommended Standard: M14 cell; LNCaP cell; JURKAT cell; (Standards are recommendations only. Compatibility of the standards in the user developed application needs to be established and confirmed by the researcher.
• Storage Buffer: Capture: 50% Glycerol, 0.01M PBS, PH 7.4; Detection: 50% Glycerol, 0.01M PBS, PH 7.4
• Other Note: Immunogen information is company proprietary information. Antibodies are sold as a pair and not available separately.
• Preparation and Storage: Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze/thaw cycles. When stored properly, products should be stable for at least 5 months.

NCBI and Uniprot Product Information

• NCBI GI #: 300192933
• NCBI GeneID: 10939
• NCBI Accession #: NP_006787.2
• NCBI GenBank Nucleotide #: NM_006796.2
• 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.-; Membrane protein, multi-pass; Chaperone; Mitochondrial; Protease; Cell development/differentiation; Membrane protein, integral

Chromosomal Location of Human Ortholog: 18p11

Cellular Component: integral to membrane; mitochondrial inner membrane; mitochondrion

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

Biological Process: axonogenesis; cristae formation; mitochondrial fusion; muscle fiber development; myelination; nerve development; neuromuscular junction development; protein complex assembly; protein import into mitochondrial intermembrane space; regulation of multicellular organism growth; righting reflex

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