Anti-AFG3L2 Monoclonal Antibody for WB, IP, IF-P, IHC-P, ELISA - Q9Y4W6

Core Product Specifications and Parameters

Biological Background: AFG3L2 Function and Localization

• AFG3L2 (AFG3-like protein 2, also known as paraplegin-like protein) is a mitochondrial inner membrane AAA+ protease that functions as the catalytic component of the m-AAA protease complex, essential for maintaining proteostasis of inner membrane proteins and critical for axonal and neuronal development. Related references: PMID:19748354, PMID:28396416, PMID:29932645
• It mediates quality control by degrading misfolded or mistranslated polypeptides in the inner mitochondrial membrane, preventing proteotoxic stress. Related references: PMID:26504172, PMID:30683687, PMID:34718584
• AFG3L2 is responsible for the proteolytic maturation of several mitochondrial proteins, including the ribosomal subunit MRPL32/bL32m, the kinase PINK1, and the metalloprotease SPG7. Related references: PMID:22354088, PMID:29932645, PMID:30252181
• It regulates mitochondrial calcium uptake by degrading SMDT1/EMRE, a component of the uniporter complex, thereby influencing cellular calcium signaling. Related references: PMID:27642048, PMID:28396416
• AFG3L2 modulates glutathione homeostasis via iron-sulfur-sensitive cleavage and degradation of the mitochondrial transporter SLC25A39. Related references: PMID:37917749, PMID:38157846
• It also participates in carnitine biosynthesis by processing SLC25A45, and in OPA1 processing through regulation of OMA1. Related references: PMID:41075794, PMID:17615298, PMID:29545505, PMID:30252181
• Ubiquitously expressed, with notably high expression in cerebellar Purkinje cells, localizing exclusively to the mitochondrial inner membrane.
• Belongs to the AAA+ ATPase family and contains sites for ATP binding, zinc coordination, and proteolytic activity; mutations are linked to spinocerebellar ataxia type 28 and spastic ataxia 5.

Experimental Guidance and Technical Tips

• The immunogen corresponds to the C-terminal region (aa 538–797); it is expected to recognize full-length AFG3L2 and potentially any C-terminal containing isoforms or cleavage products.
• For Western blotting, a predominant band near 89 kDa is anticipated. Validate in lysates from human, mouse, or rat cell lines or tissues known to express AFG3L2, such as brain or muscle.
• In immunoprecipitation, the antibody can be used to enrich AFG3L2 complexes; consider cross-linking to protein A/G beads for best results.
• For immunofluorescence and immunohistochemistry, a mitochondrial staining pattern is expected; co-staining with a mitochondrial marker is recommended for confirmation.
• ELISA may be performed using direct coating of recombinant AFG3L2 protein or capture format; titrate antibody for optimal signal.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio partners with diagnostic developers and researchers to supply high-quality IVD raw materials. This anti-AFG3L2 monoclonal antibody supports studies of mitochondrial proteases and related neurodegenerative diseases, and can be integrated into your assay development pipeline. Beyond single antibodies, CamelBio offers validated antibody pairs, bulk ancillary reagents, and sourcing for rare target raw materials—helping you move from concept to clinic efficiently.