製品の概要

  • 製品名
  • 製品の詳細
    Goat polyclonal to Als2
  • アプリケーション
    適用あり: IHC-Pmore details
  • 種交差性
    交差種: Human
    交差が予測される動物種: Mouse, Rat, Chimpanzee
  • 免疫原

    Synthetic peptide:

    LKACYYQIQREKLN

    , corresponding to C terminal amino acids 1644-1657 of Human Als2 (NP_065970.2).

  • ポジティブ・コントロール
    • Human brain cortex tissue

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab123515 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

アプリケーション Abreviews 特記事項
IHC-P Use a concentration of 5 - 10 µg/ml. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

ターゲット情報

  • 機能
    May act as a GTPase regulator. Controls survival and growth of spinal motoneurons.
  • 関連疾患
    Defects in ALS2 are the cause of amyotrophic lateral sclerosis type 2 (ALS2) [MIM:205100]. ALS2 is a familial form of amyotrophic lateral sclerosis, a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of cases leading to familial forms.
    Defects in ALS2 are the cause of juvenile primary lateral sclerosis (JPLS) [MIM:606353]. JPLS is a neurodegenerative disorder which is closely related to but clinically distinct from amyotrophic lateral sclerosis. It is a progressive paralytic disorder which results from dysfunction of the upper motor neurons of the motor cortex while the lower neurons are unaffected.
    Defects in ALS2 are the cause of infantile-onset ascending spastic paralysis (IAHSP) [MIM:607225]. IAHSP is characterized by progressive spasticity and weakness of limbs.
  • 配列類似性
    Contains 1 DH (DBL-homology) domain.
    Contains 8 MORN repeats.
    Contains 1 PH domain.
    Contains 5 RCC1 repeats.
    Contains 1 VPS9 domain.
  • 翻訳後修飾
    Phosphorylated upon DNA damage, probably by ATM or ATR.
  • Information by UniProt
  • 参照データベース
  • 別名
    • ALS 2 antibody
    • ALS2 antibody
    • ALS2_HUMAN antibody
    • ALS2CR6 antibody
    • Alsin antibody
    • ALSJ antibody
    • Amyotrophic lateral sclerosis 2 (juvenile) antibody
    • Amyotrophic lateral sclerosis 2 (juvenile) chromosome region candidate 6 antibody
    • Amyotrophic lateral sclerosis 2 chromosomal region candidate gene 6 protein antibody
    • Amyotrophic lateral sclerosis 2 protein antibody
    • Amyotrophic lateral sclerosis protein 2 antibody
    • FLJ31851 antibody
    • IAHSP antibody
    • KIAA1563 antibody
    • MGC87187 antibody
    • PLSJ antibody
    see all

画像

  • ab123515, at 5 µg/ml, staining Als2 in formalin fixed, paraffin embedded Human brain cortex tissue by Immunohistochemistry.

プロトコール

参考文献

ab123515 has not yet been referenced specifically in any publications.

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