Overview

  • Product name
  • Description
    Goat polyclonal to Als2
  • Tested applications
    Suitable for: IHC-Pmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

    Synthetic peptide:

    LKACYYQIQREKLN

    , corresponding to C terminal amino acids 1644-1657 of Human Als2.

  • General notes
    Principal Names - ALS2; ALSJ; PLSJ; IAHSP; KIAA1563; amyotrophic lateral sclerosis 2 (juvenile) Official Gene Symbol - ALS2 GenBank Accession Number – NP_065970 LocusLink ID - 57679 (human)

Properties

Applications

Our Abpromise guarantee covers the use of ab4155 in the following tested applications.

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

Application Abreviews Notes
IHC-P Use a concentration of 3 - 5 µg/ml.

Target

  • Function
    May act as a GTPase regulator. Controls survival and growth of spinal motoneurons.
  • Involvement in disease
    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.
  • Sequence similarities
    Contains 1 DH (DBL-homology) domain.
    Contains 8 MORN repeats.
    Contains 1 PH domain.
    Contains 5 RCC1 repeats.
    Contains 1 VPS9 domain.
  • Post-translational
    modifications
    Phosphorylated upon DNA damage, probably by ATM or ATR.
  • Information by UniProt
  • Database links
  • Alternative names
    • 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

Images

  • ab4155 at 3.8µg/ml staining Als2 in human cortex tissue section by Immunohistochemistry (Formalin/ PFA-fixed paraffin embedded tissue sections). The tissue underwent antigen retrieval by steam in citrate buffer at pH 6.0. The AP-staining procedure was used for detection. 

References

ab4155 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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