Description

Specifications

Our Abpromise guarantee covers the use of ab82649 in the following tested applications.

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

  • Applications

    SDS-PAGE

    Western blot

  • Form

    Lyophilised
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Upon delivery aliquot. Store at -80°C. Avoid freeze / thaw cycle.

    pH: 7.40
    Constituent: 0.476% HEPES

General Info

  • Alternative names

    • ALS
    • ALS1
    • Amyotrophic lateral sclerosis 1 adult
    • Cu/Zn SOD
    • Cu/Zn superoxide dismutase
    • Epididymis secretory protein Li 44
    • HEL S 44
    • Homodimer
    • hSod1
    • Indophenoloxidase A
    • IPOA
    • Mn superoxide dismutase
    • SOD
    • SOD soluble
    • SOD1
    • SOD2
    • SODC
    • SODC_HUMAN
    • Superoxide dismutase [Cu-Zn]
    • Superoxide dismutase 1
    • Superoxide dismutase 1 soluble
    • Superoxide dismutase Cu Zn
    • Superoxide dismutase cystolic
    see all
  • Function

    Destroys radicals which are normally produced within the cells and which are toxic to biological systems.
  • Involvement in disease

    Defects in SOD1 are the cause of amyotrophic lateral sclerosis type 1 (ALS1) [MIM:105400]. ALS1 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.
  • Sequence similarities

    Belongs to the Cu-Zn superoxide dismutase family.
  • Post-translational
    modifications

    Unlike wild-type protein, the pathogenic variants ALS1 Arg-38, Arg-47, Arg-86 and Ala-94 are polyubiquitinated by RNF19A leading to their proteasomal degradation. The pathogenic variants ALS1 Arg-86 and Ala-94 are ubiquitinated by MARCH5 leading to their proteasomal degradation.
    The ditryptophan cross-link at Trp-33 is reponsible for the non-disulfide-linked homodimerization. Such modification might only occur in extreme conditions and additional experimental evidence is required.
  • Cellular localization

    Cytoplasm. The pathogenic variants ALS1 Arg-86 and Ala-94 gradually aggregates and accumulates in mitochondria.
  • Information by UniProt

Images

  • ab82649 (1µg) on SDS-PAGE.

References

ab82649 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

Answer

Thank you for your enquiry which has been forwarded to the scientific support team (mailto:technical@abcam.com).

I am sorry to confirm that because we carry over 70,000 products, regrettably it is not feasible for us to keep small sample sizes of our products to send to customers. Unfortunately we do not sell these proteins in smaller amounts.

We are happy to reassure our customers that all of our products are covered by our Abpromise, which guarantees that the product will work in the applications specified on the datasheet, or we will offer a replacement, credit, or refund within 6 months of purchase.

These proteins have been tested in western blot, and are not guaranteed for other applications.

With regards to quotatation, the price can be found on the online datasheets. You will need to ensure the correct country is selected from the top right hand corner of the page in order to see the correct price. I can confirm the following:

ab82649
500ug £180.00

ab88460
100ul £227.00

ab74916
100ug £180.00

Delivery charge in UK: £16.00

In this case, as a gesture of goodwill we would be pleased towaive the delivery charge if you decide to purchase all three peptides. Please let me know by replyingdirectly to this email, and I can provide you with a code which you will need to provide with your order.

Also, we can offer a discount for any purchases of 5 items or more. In this case, you will need to contact our customer service department (mailto:orders@abcam.com) with a list of what you want to order, and they can provide you with a quote.

I hope this information is helpful. If you have any further questions, or require any further information regarding these proteins, please do not hesitate to contact me.

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Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
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