Overview

  • Product name

    Anti-ATPAF2 antibody
  • Description

    Rabbit polyclonal to ATPAF2
  • Host species

    Rabbit
  • Specificity

    This antibody detects endogenous levels of total ATPAF2 protein.
  • Tested applications

    Suitable for: ELISA, WBmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse
  • Immunogen

    A synthesized peptide derived from the internal region of human ATPAF2.

  • Positive control

    • Extracts from Jurkat cells.

Properties

Applications

Our Abpromise guarantee covers the use of ab74235 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
ELISA 1/40000.
WB 1/500 - 1/1000. Detects a band of approximately 33 kDa (predicted molecular weight: 33 kDa).

Target

  • Relevance

    ATPAF2 belongs to the ATP12 family. It may play a role in the assembly of the F1 component of the mitochondrial ATP synthase (ATPase). Defects in ATPAF2 are the cause of complex V mitochondrial respiratory chain ATPAF2 subunit deficiency (ATPAF2 deficiency) [MIM:604273]; also called ATP synthase deficiency or ATPase deficiency. ATPAF2 deficiency seems to be an early presenting disease in which lactic acidosis, dysmorphic features, and methyl glutaconic aciduria can be major clues in the diagnosis. Dysmorphic features include a large mouth, prominent nasal bridge, micrognathia, rocker-bottom feet and flexion contractures of the limbs associated with camptodactyly. Patients are hypertonic and have an enlarged liver, hypoplastic kidneys and elevated lactate levels in urine, plasma and cerebro spinal fluid (CSF).
  • Cellular localization

    Mitochondrion
  • Database links

  • Alternative names

    • ATP synthase mitochondrial F1 complex assembly factor 2 antibody
    • ATP12 antibody
    • ATP12 homolog antibody
    • ATP12p antibody
    • LP3663 antibody
    • MC5DN1 antibody
    see all

Images

  • All lanes : Anti-ATPAF2 antibody (ab74235) at 1/500 dilution

    Lane 1 : Extracts from Jurkat cells
    Lane 2 : Extracts from Jurkat cells plus 5µg immunizing peptide

    Lysates/proteins at 5 µg per lane.

    Predicted band size: 33 kDa
    Observed band size: 33 kDa

References

ab74235 has not yet been referenced specifically in any publications.

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