Mitofusin 2 Rabbit Polyclonal Antibody (ER1802-23)

UNIPROT #

O95140

PROTEIN NAME

Mitofusin 2

SYNONYMS

CMT2A antibody;CMT2A2 antibody;CPRP 1 antibody;CPRP1 antibody;EC 3.6.5.- antibody;Fzo antibody;HSG antibody;hyperplasia suppressor gene antibody;Hypertension related protein 1 antibody;KIAA0214 antibody;MARF antibody;MFN 2 antibody;Mfn2 antibody;MFN2_HUMAN antibody;Mitochondrial assembly regulatory factor antibody;Mitofusin-2 antibody;Mitofusin2 antibody;Transmembrane GTPase MFN2 antibody

SEQUENCE SIMILARITIES

Belongs to the TRAFAC class dynamin-like GTPase superfamily. Dynamin/Fzo/YdjA family. Mitofusin subfamily.

TISSUE SPECIFICITY

Ubiquitous; expressed at low level. Highly expressed in heart and kidney.

POST-TRANSLATIONAL MODIFICATION

Phosphorylated by PINK1.; Ubiquitinated by non-degradative ubiquitin by PRKN, promoting mitochondrial fusion; deubiquitination by USP30 inhibits mitochondrial fusion.

SUBCELLULAR LOCATION

Mitochondrion.

FUNCTION

Mitofusin 1 (Mfn1) and mitofusin 2 (Mfn2) are homologs for the Drosophila protein fuzzy onion (Fzo). They are mitochondrial membrane proteins and are mediators of mitochondrial fusion. A GTPase domain is required for Mfn protein function but the molecular mechanisms of the GTPase-dependent reaction as well as the functional division of the two Mfn proteins are unknown. They are essential for embryonic development and may play a role in the pathobiology of obesity. Although the Mfn1 and Mfn2 genes are broadly expressed, they show different levels of expression in different tissues. Two Mfn1 transcripts are elevated in heart, while Mfn2 mRNA is abundantly expressed in heart and muscle tissue but present only at low levels in many other tissues. Mfn1 localizes to mitochondria and participates in at least two different high molecular weight protein complexes in a GTP-dependent manner. Purified recombinant Mfn1 exhibited approximately eightfold higher GTPase activity than Mfn2.