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| Product Name
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AFG3-like protein 2 (AFG3L2), Polyclonal Antibody
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| Also Known As
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Rabbit anti-human AFG3-like protein 2 polyclonal Antibody, FITC
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| Product Synonym Names
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Paraplegin-like protein
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| Product Gene Name
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[Similar Products]
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| Research Use Only
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For Research Use Only. Not for use in diagnostic procedures.
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 TOP
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| OMIM
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604581
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| 3D Structure
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ModBase 3D Structure for Q9Y4W6
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| Clonality
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Polyclonal
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| Isotype
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IgG
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| Host
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Rabbit
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| Species Reactivity
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Human
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| Purity/Purification
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Caprylic Acid Ammonium Sulfate Precipitation Purified
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| Storage Buffer
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Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
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| Conjugate
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FITC
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| Immunogen
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Recombinant human AFG3-like protein 2 protein
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| Santa Cruz Alternative
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Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-84686 / sc-84687
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| Preparation and Storage
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Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.
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| ISO Certification
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Manufactured in an ISO 9001:2008 Certified Laboratory.
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| Other Notes
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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.
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TOP
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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.
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| Applications Tested/Suitable for anti-AFG3L2 antibody
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ELISA (EIA)
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| Testing Data of anti-AFG3L2 antibody
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TOP
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| NCBI/Uniprot data below describe general gene information for AFG3L2. It may not necessarily be applicable to this product. |
| NCBI GI #
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300192933
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| NCBI GeneID
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10939
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| NCBI Accession #
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NP_006787.2
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[Other Products]
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| NCBI GenBank Nucleotide #
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NM_006796.2
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[Other Products]
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| UniProt Primary Accession #
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Q9Y4W6
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[Other Products]
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| UniProt Secondary Accession #
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Q6P1L0
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[Other Products]
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| UniProt Related Accession #
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Q9Y4W6
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[Other Products]
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| Molecular Weight
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88,584 Da
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TOP
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| NCBI Official Full Name
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AFG3-like protein 2
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| NCBI Official Synonym Full Names
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AFG3-like AAA ATPase 2
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| NCBI Official Symbol
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AFG3L2
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[Similar Products]
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| NCBI Official Synonym Symbols
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SCA28; SPAX5
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[Similar Products]
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| NCBI Protein Information
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AFG3-like protein 2
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| UniProt Protein Name
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AFG3-like protein 2
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| UniProt Synonym Protein Names
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Paraplegin-like protein
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| Protein Family
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AFG3-like protein
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| UniProt Gene Name
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AFG3L2
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[Similar Products]
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| UniProt Entry Name
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AFG32_HUMAN
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| NCBI Summary for AFG3L2
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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]
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| UniProt Comments for AFG3L2
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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; Protease; Chaperone; Mitochondrial; 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
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TOP
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| Research Articles on AFG3L2
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1. Deletion of AFG3L2 associated with spinocerebellar ataxia type 28 in the context of multiple genomic anomalies.
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TOP
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| Precautions
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All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
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| Disclaimer
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While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.
It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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| Products associated with anti-AFG3L2 antibody | Diseases associated with anti-AFG3L2 antibody |
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| Organs/Tissues associated with anti-AFG3L2 antibody |
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Antibody 
