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Anti-AFG3L2 Antibody Picoband® Fluoro594 Conjugated

Product Specifications

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.

Synonyms

AFG3-like protein 2;3.4.24.-; Paraplegin-like protein; AFG3L2

Gene Name

AFG3L2

Gene ID

10939

UniProt

Q9Y4W6

Host

Rabbit

Reactivity

Human, Mouse, Rat

Cross Reactivity

No cross-reactivity with other proteins

Immunogen

E. coli-derived human AFG3L2 recombinant protein (Position: R168-D250) . Human AFG3L2 shares 100% amino acid (aa) sequence identity with mouse AFG3L2.

Clonality

Polyclonal

Tissue Specificity

Ubiquitous. Highly expressed in the cerebellar Purkinje cells. .

Applications

Flow Cytometry

Field of Research

Cell Biology, Metabolism, Mitochondrial, Mitochondrial Markers, Mitochondrial Metabolism, Neurodegenerative Disease, Neurology Process, Neuroscience, Pathways and Processes, Proteolysis/Ubiquitin, Proteolytic Enzymes, Signal Transduction

Purification

Immunogen affinity purified.

Form

Liquid

Function

ATP-dependent protease which is essential for axonal development. .

References & Citations

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.

Storage Conditions

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

Calculated Molecular Weight

88584 MW

Specificity

No cross reactivity with other proteins.

Applications Notes

6

Gene Name Synonym

AFG3-like protein 2

Subcellular Location

Mitochondrion membrane ; Multi-pass membrane protein .

Protein Name

AFG3-like protein 2

Isotype

Rabbit IgG

Contents

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.

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