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AFG3L2 抗体 (AA 168-250)

This anti-AFG3L2 antibody is a 兔 多克隆 antibody detecting AFG3L2 in WB, IF, ICC 和 FACS. Suitable for 人, 小鼠 和 大鼠.
产品编号 ABIN4886437
发货至: 中国

Quick Overview for AFG3L2 抗体 (AA 168-250) (ABIN4886437)

抗原

See all AFG3L2 抗体
AFG3L2 (AFG3-Like Protein 2 (AFG3L2))

适用

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人, 小鼠, 大鼠

宿主

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克隆类型

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多克隆

标记

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This AFG3L2 antibody is un-conjugated

应用范围

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Western Blotting (WB), Immunofluorescence (IF), Immunocytochemistry (ICC), Flow Cytometry (FACS)
  • 抗原表位

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    AA 168-250

    原理

    Anti-AFG3L2 Antibody Picoband®

    交叉反应 (详细)

    No cross-reactivity with other proteins

    产品特性

    Anti-AFG3L2 Antibody Picoband® (ABIN4886437). Tested in Flow Cytometry, IF, ICC, WB applications. This antibody reacts with Human, Mouse, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

    纯化方法

    Immunogen affinity purified.

    免疫原

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

    亚型

    IgG
  • 应用备注

    Western blot, 0.1-0.5 μg/mL, Human, Mouse, Rat
    Immunocytochemistry/Immunofluorescence, 2 μg/mL, Human
    Flow Cytometry (Fixed), 1-3 μg/1x106 cells, Human1. "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.

    说明

    Antibody can be supported by chemiluminescence kit ABIN921124 in WB.

    限制

    仅限研究用
  • 状态

    Lyophilized

    溶解方式

    Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.

    浓度

    500 μg/mL

    缓冲液

    Each vial contains 5 mg BSA, 0.9 mg NaCl, 0.2 mg Na2HPO4, 0.05 mg Sodium azide.

    储存液

    Sodium azide

    注意事项

    This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

    注意事项

    Avoid repeated freezing and thawing.

    储存条件

    4 °C,-20 °C

    储存方法

    Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month.
    It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.
  • 抗原

    AFG3L2 (AFG3-Like Protein 2 (AFG3L2))

    别名

    AFG3L2

    背景

    Synonyms: AFG3-like protein 2,3.4.24.-,Paraplegin-like protein,AFG3L2,

    Tissue Specificity: Ubiquitous. Highly expressed in the cerebellar Purkinje cells. .

    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.

    分子量

    89 kDa

    基因ID

    10939

    UniProt

    Q9Y4W6

    途径

    Skeletal Muscle Fiber Development
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