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

Scan QR to view Datasheet Catalog #    MBS715066
Testing Data
Unit / Price
0.05 mg  /  $160 +1 FREE 8GB USB
0.1 mg  /  $235 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

AFG3-like protein 2 (AFG3L2), Polyclonal Antibody

 Also Known As   

Rabbit anti-human AFG3-like protein 2 polyclonal Antibody, Biotin conjugated

 Product Synonym Names    Paraplegin-like protein
 Product Gene Name   

anti-AFG3L2 antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    604581
 3D Structure    ModBase 3D Structure for Q9Y4W6
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 Clonality    Polyclonal
 Isotype    IgG
 Host    Rabbit
 Species Reactivity    Human
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 Purity/Purification    Caprylic Acid Ammonium Sulfate Precipitation Purified
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 Storage Buffer    Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
 Conjugate    Biotin
 Immunogen    Recombinant human AFG3-like protein 2 protein
 Santa Cruz Alternative    Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-84686 / sc-84687
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 Preparation and Storage    Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.
 ISO Certification    Manufactured in an ISO 9001:2008 Certified Laboratory.
 Other Notes    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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Product Description specifically 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.
 Applications Tested/Suitable for anti-AFG3L2 antibody   

ELISA (EIA)

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 Testing Data of anti-AFG3L2 antibody    anti-AFG3L2 antibody Testing Data image
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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 #    300192933
 NCBI GeneID    10939
 NCBI Accession #    NP_006787.2 [Other Products]
 NCBI GenBank Nucleotide #    NM_006796.2 [Other Products]
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 UniProt Primary Accession #    Q9Y4W6 [Other Products]
 UniProt Secondary Accession #    Q6P1L0 [Other Products]
 UniProt Related Accession #    Q9Y4W6 [Other Products]
 Molecular Weight    88,584 Da
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 NCBI Official Full Name    AFG3-like protein 2
 NCBI Official Synonym Full Names    AFG3-like AAA ATPase 2
 NCBI Official Symbol    AFG3L2 [Similar Products]
 NCBI Official Synonym Symbols   
SCA28; SPAX5
[Similar Products]
 NCBI Protein Information    AFG3-like protein 2
 UniProt Protein Name    AFG3-like protein 2
 UniProt Synonym Protein Names   
Paraplegin-like protein
 Protein Family    AFG3-like protein
 UniProt Gene Name    AFG3L2 [Similar Products]
 UniProt Entry Name    AFG32_HUMAN
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 NCBI Summary for AFG3L2    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    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: Protease; Membrane protein, multi-pass; EC 3.4.24.-; Mitochondrial; Chaperone; 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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 Research Articles on AFG3L2    1. Deletion of AFG3L2 associated with spinocerebellar ataxia type 28 in the context of multiple genomic anomalies.
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 Precautions    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.
 Disclaimer    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 antibodyDiseases associated with anti-AFG3L2 antibody
 Reference Product  PubMed Publications
 OMA1 antibody  >2 publications with AFG3L2 and OMA1
 YME1L1 antibody  >1 publications with AFG3L2 and YME1L1
 Disease Name  Pubmed Publications
 Spinocerebellar Ataxias Antibodies  >19 publications with AFG3L2 and Spinocerebellar Ataxias
 Spinocerebellar ataxia 28 Antibodies  >11 publications with AFG3L2 and Spinocerebellar ataxia 28
 Nervous System Malformations Antibodies  >9 publications with AFG3L2 and Nervous System Malformations
 Atrophy Antibodies  >7 publications with AFG3L2 and Atrophy
 Disease Models, Animal Antibodies  >3 publications with AFG3L2 and Disease Models, Animal
 Embryo Loss Antibodies  >2 publications with AFG3L2 and Embryo Loss
 Nerve Degeneration Antibodies  >2 publications with AFG3L2 and Nerve Degeneration
 Neurobehavioral Manifestations Antibodies  >1 publications with AFG3L2 and Neurobehavioral Manifestations
 Abnormalities, Multiple Antibodies  >1 publications with AFG3L2 and Abnormalities, Multiple
 Myoclonic Epilepsies, Progressive Antibodies  >1 publications with AFG3L2 and Myoclonic Epilepsies, Progressive
Organs/Tissues associated with anti-AFG3L2 antibody
 Organ/Tissue Name  Pubmed Publications
 Brain Antibodies  >17 publications with AFG3L2 and Brain
 Nerve Antibodies  >6 publications with AFG3L2 and Nerve
 Muscle Antibodies  >4 publications with AFG3L2 and Muscle
 Embryonic Tissue Antibodies  >2 publications with AFG3L2 and Embryonic Tissue
 Eye Antibodies  >2 publications with AFG3L2 and Eye
 Bone Antibodies  >1 publications with AFG3L2 and Bone
 Heart Antibodies  >1 publications with AFG3L2 and Heart
 Ovary Antibodies  >1 publications with AFG3L2 and Ovary
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