Mtx2-flox Mouse

MTX2, encoding Metaxin-2, is an outer mitochondrial membrane protein. It is involved in maintaining mitochondrial cristae architecture, which is crucial for preventing mitochondrial DNA (mtDNA) release and subsequent activation of the type I interferon (IFN-I) response via the cGAS-STING pathway [1]. It also plays roles in various cellular processes and is associated with multiple diseases.

In mouse models, conditional podocyte-specific Mtx2 knockout (Pod-Mtx2-KO) mice show a series of podocyte and glomerular abnormalities, including microalbuminuria, glomerular mesangial hyperplasia, and foot process fusion. MTX2 deficiency impairs podocyte functions like adhesion, migration, and endocytosis, accompanied by abnormal mitochondrial structure and dysfunction [2]. Knocking out MTX2 in mouse liver induces a robust STING-dependent IFN-I response, highlighting its role in inflammation regulation [1]. In zebrafish, morpholino knockdown of Mtx2, a homeobox transcription factor, results in stalling of epiboly and yolk rupture due to its role in regulating microfilament formation during morphogenetic movements [3].

In conclusion, MTX2 is essential for maintaining mitochondrial structure and function, and its proper expression is crucial for normal cellular functions in podocytes and other cell types. The study of MTX2 knockout models has revealed its significance in diseases such as glomerular diseases, inflammation-related disorders, and mandibuloacral dysplasia progeroid syndrome [1,3,5-7].