The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 0.2 - 1 µg/ml. Predicted molecular weight: 82 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
Use a concentration of 2 µg/ml.
機能Functions in mitochondrial and peroxisomal division. Mediates membrane fission through oligomerization into ring-like structures which wrap around the scission site to constict and sever the mitochondrial membrane through a GTP hydrolysis-dependent mechanism. Required for normal brain development. Facilitates developmentally-regulated apoptosis during neural tube development. Required for a normal rate of cytochrome c release and caspase activation during apoptosis. Also required for mitochondrial fission during mitosis. May be involved in vesicle transport. Isoform 1 and isoform 4 inhibit peroxisomal division when overexpressed.
組織特異性Ubiquitously expressed with highest levels found in skeletal muscles, heart, kidney and brain. Isoform 1 is brain-specific. Isoform 2 and isoform 3 are predominantly expressed in testis and skeletal muscles respectively. Isoform 4 is weakly expressed in brain, heart and kidney. Isoform 5 is dominantly expressed in liver, heart and kidney. Isoform 6 is expressed in neurons.
関連疾患Note=May be associated with Alzheimer disease through beta-amyloid-induced increased S-nitrosylation of DNM1L, which triggers, directly or indirectly, excessive mitochondrial fission, synaptic loss and neuronal damage.
配列類似性Belongs to the dynamin family. Contains 1 GED domain.
ドメインThe GED domain folds back to interact, in cis, with the GTP-binding domain and middle domain, and interacts, in trans, with the GED domains of other DNM1L molecules, and is thus critical for activating GTPase activity and for DNM1L dimerization.
翻訳後修飾Phosphorylation/dephosphorylation events on two sites near the GED domain regulate mitochondrial fission. Phosphorylation on Ser-637 inhibits mitochondrial fissin probably through preventing intramolecular interaction. Dephosphorylated on this site by PPP3CA which promotes mitochondrial fission. Phosphorylation on Ser-616 also promotes mitochondrial fission. Sumoylated on various lysine residues within the B domain. Desumoylated by SENP5 during G2/M transition of mitosis. Appears to be linked to its catalytic activity. S-nitrosylation increases DNM1L dimerization, mitochondrial fission and causes neuronal damage. Ubiquitination by MARCH5 affects mitochondrial morphology.
細胞内局在Cytoplasm > cytosol. Golgi apparatus. Endomembrane system. Mainly cytosolic. Translocated to the mitochondrial membrane through interaction with FIS1. Colocalized with MARCH5 at mitochondrial membrane. Localizes to mitochondria at sites of division. Associated with peroxisomal membranes, partly recruited there by PEX11B. May also be associated with endoplasmic reticulum tubules and cytoplasmic vesicles and found to be perinuclear. In some cell types, localizes to the Golgi complex.