Anti-AFG3L2 (OPA12/SCA28/SPAX5) Monoclonal Antibody for WB, IHC-P, IF/ICC - Q9Y4W6

Core Product Specifications and Parameters

Biological Background: AFG3L2 Function and Localization

• AFG3L2, also known as AFG3-like protein 2 or paraplegin-like protein, is a catalytic component of the mitochondrial inner membrane m-AAA protease, essential for axonal and neuronal development.
  Related references: PMID:19748354 PMID:28396416 PMID:29932645
• It possesses dual ATPase and protease activities, using ATP hydrolysis to unfold substrates and thread them into the proteolytic cavity for degradation.
  Related references: PMID:19748354 PMID:31327635
• The m-AAA protease ensures mitochondrial protein quality control by degrading misfolded or mistranslated inner membrane proteins.
  Related references: PMID:26504172 PMID:30683687 PMID:34718584
• AFG3L2 mediates maturation of critical mitochondrial proteins, including MRPL32, PINK1, and SP7, by cleaving their presequences.
  Related references: PMID:22354088 PMID:29932645 PMID:30252181
• It regulates calcium homeostasis in neurons by degrading SMDT1/EMRE, thereby limiting MCU assembly and mitochondrial calcium uptake.
  Related references: PMID:27642048 PMID:28396416
• AFG3L2 also controls mitochondrial glutathione levels through cleavage of the transporter SLC25A39, a process inhibited by iron-sulfur cluster binding.
  Related references: PMID:37917749 PMID:38157846
• Localized to the mitochondrial inner membrane, AFG3L2 is ubiquitously expressed with high levels in cerebellar Purkinje cells, and mutations are associated with spinocerebellar ataxia type 28 (SCA28) and spastic ataxia 5 (SPAX5).

Experimental Guidance and Technical Tips

• The immunogen encompasses the C-terminal region (aa 538–797), which may enable detection of full-length AFG3L2 as well as processed fragments.
• For Western blotting, an expected band near 89 kDa can be observed; enrichment of mitochondrial fractions may improve signal intensity.
• In immunohistochemistry (IHC-P), tissue fixation and antigen retrieval conditions should be optimized for each sample type.
• Given cross-reactivity with mouse and rat, the antibody can be used across common model organisms; validate in the relevant tissue or cell line.
• For immunofluorescence (IF/ICC), permeabilization steps are necessary to access the mitochondrial inner membrane where AFG3L2 resides.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio supports diagnostic manufacturers and research institutes with a comprehensive portfolio of IVD raw materials, including validated antibodies for targets like AFG3L2—a mitochondrial protease linked to neurodegeneration. We simplify procurement by offering optimized monoclonal/polyclonal antibodies, bulk ancillary reagents, and rare target sourcing, backed by technical consultation. Whether you need a single reagent or an integrated solution, CamelBio serves as your strategic partner from concept to clinical application.