製品の概要

製品の詳細

  • 由来
    Synthetic
  • アミノ酸配列
    • 生物種
      Human
    • 配列
      LKACYYQIQREKLN
    • 領域
      1644 to 1657

関連製品

特性

Our Abpromise guarantee covers the use of ab22998 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

    Blocking - Blocking peptide for Anti-Als2 antibody (ab4155)

  • 製品の状態
    Liquid
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

関連情報

  • 別名
    • ALS 2
    • ALS2
    • ALS2_HUMAN
    • ALS2CR6
    • Alsin
    • ALSJ
    • Amyotrophic lateral sclerosis 2 (juvenile)
    • Amyotrophic lateral sclerosis 2 (juvenile) chromosome region candidate 6
    • Amyotrophic lateral sclerosis 2 chromosomal region candidate gene 6 protein
    • Amyotrophic lateral sclerosis 2 protein
    • Amyotrophic lateral sclerosis protein 2
    • FLJ31851
    • IAHSP
    • KIAA1563
    • MGC87187
    • PLSJ
    see all
  • 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

参考文献

ab22998 has not yet been referenced specifically in any publications.

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