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Rabbit Anti-Mitofusin 1/FITC Conjugated antibody
background:
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.
Function:
Essential transmembrane GTPase, which mediates mitochondrial fusion. Fusion of mitochondria occurs in many cell types and constitutes an important step in mitochondria morphology, which is balanced between fusion and fission. MFN1 acts independently of the cytoskeleton. Overexpression induces the formation of mitochondrial networks.
Subunit:
Forms homomultimers and heteromultimers with MFN2. Multimerization, which is mediated by the second coiled coil region, may play an essential role in mitochondrion fusion. Participates in a high molecular weight multiprotein complex. Interacts with VAT1.
Subcellular Location:
Cytoplasm and Mitochondrion outer membrane.
Tissue Specificity:
Ubiquitous. Expressed at slightly higher level in kidney and heart. Isoform 2 may be overexpressed in some tumors, such as lung cancers.
Post-translational modifications:
Ubiquitinated by MARCH5.
Similarity:
Belongs to the mitofusin family.
Database links:
Entrez Gene: 55669 Human
Entrez Gene: 67414 Mouse
Entrez Gene: 192647 Rat
Omim: 608506 Human
SwissProt: Q8IWA4 Human
SwissProt: Q811U4 Mouse
SwissProt: Q8R4Z9 Rat
Unigene: 478383 Human
Unigene: 290414 Mouse
Unigene: 160939 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
线粒体融合蛋白1具有促进线粒体融合、抑制细胞增殖、保护细胞免于凋亡等多种功能。
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