DNM1L

Chr 12p11.21

dynamin 1L

Aliases:: DRP1, DVLP, HDYNIV, DYMPLE, VPS1
MANE:: ENST00000549701.6

OMIM: 603850 ↗NCBI: 10059 ↗Ensembl ↗

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Predicted protein structure

AlphaFold predicted structure

DNM1L · O00429

v62025-08-01

Predicted Aligned Error matrix for DNM1L — diagonal shows residue index, colour shows pairwise prediction confidence (lower is better) — Predicted Aligned Error

Mean pLDDT

76.4/ 100

Confident

736 residues

Confidence breakdown

Very high(≥ 90)31%
Confident(70–90)42%
Low(50–70)10%
Very low(< 50)17%

Open interactive 3D viewer

AlphaFold (Jumper et al., 2021) · CC BY 4.0

Clinical relevance (Genomics England PanelApp)

Diagnostic Grade (Green)

DDG2P
MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Early onset or syndromic epilepsy
BOTH monoallelic and biallelic, autosomal or pseudoautosomal
Fetal anomalies
BOTH monoallelic and biallelic, autosomal or pseudoautosomal
Intellectual disability
BOTH monoallelic and biallelic, autosomal or pseudoautosomal
Likely inborn error of metabolism
BOTH monoallelic and biallelic, autosomal or pseudoautosomal
Mitochondrial disorders
BOTH monoallelic and biallelic, autosomal or pseudoautosomal
Optic neuropathy
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Peroxisomal disorders
BOTH monoallelic and biallelic, autosomal or pseudoautosomal

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Disease associations (Open Targets)

Lethal encephalopathy due to mitochondrial and peroxisomal fission defect
0.77
encephalopathy, lethal, due to defective mitochondrial peroxisomal fission 1
0.75
Autosomal dominant optic atrophy, classic type
0.72
neurodegenerative disease
0.53
encephalopathy due to mitochondrial and peroxisomal fission defect
0.45
hereditary disease
0.45
Leber hereditary optic neuropathy
0.39
mitochondrial disease
0.38
autosomal dominant optic atrophy, classic form
0.38
hereditary optic atrophy
0.37

Score is the Open Targets composite evidence score (0-1). Higher = stronger gene-disease association.

Protein function (UniProt)

Dynamin-1-like protein

Functions in mitochondrial and peroxisomal division (PubMed:11514614, PubMed:12499366, PubMed:17301055, PubMed:17460227, PubMed:17553808, PubMed:18695047, PubMed:18838687, PubMed:19342591, PubMed:19411255, PubMed:19638400, PubMed:23283981, PubMed:23530241, PubMed:23921378, PubMed:26992161, PubMed:27145208, PubMed:27145933, PubMed:27301544, PubMed:27328748, PubMed:29478834, PubMed:32439975, PubMed:32484300, PubMed:9570752, PubMed:9786947). Mediates membrane fission through oligomerization into membrane-associated tubular structures that wrap around the scission site to constrict and sever the mitochondrial membrane through a GTP hydrolysis-dependent mechanism (PubMed:23530241, PubMed:23584531, PubMed:33850055). The specific recruitment at scission sites is mediated by membrane receptors like MFF, MIEF1 and MIEF2 for mitochondrial membranes (PubMed:23283981, PubMed:23921378, PubMed:29899447). While the recruitment by the membrane receptors is GTP-dependent, the following hydrolysis of GTP induces the dissociation from the receptors and allows DNM1L filaments to curl into closed rings that are probably sufficient to sever a double membrane (PubMed:29899447). Acts downstream of PINK1 to promote mitochondrial fission in a PRKN-dependent manner (PubMed:32484300). Plays an important role in mitochondrial fission during mitosis (PubMed:19411255, PubMed:26992161, PubMed:27301544, PubMed:27328748). Through its function in mitochondrial division, ensures the survival of at least some types of postmitotic neurons, including Purkinje cells, by suppressing oxidative damage (By similarity). Required for normal brain development, including that of cerebellum (PubMed:17460227, PubMed:26992161, PubMed:27145208, PubMed:27301544, PubMed:27328748). Facilitates developmentally regulated apoptosis during neural tube formation (By similarity). Required for a normal rate of cytochrome c release and caspase activation during apoptosis; this requirement may depend upon the cell type and the physiological apoptotic cues (By similarity). Required for formation of endocytic vesicles (PubMed:20688057, PubMed:23792689, PubMed:9570752). Proposed to regulate synaptic vesicle membrane dynamics through association with BCL2L1 isoform Bcl-X(L) which stimulates its GTPase activity in synaptic vesicles; the function may require its recruitment by MFF to clathrin-containing vesicles (PubMed:17015472, PubMed:23792689). Required for programmed necrosis execution (PubMed:22265414). Rhythmic control of its activity following phosphorylation at Ser-637 is essential for the circadian control of mitochondrial ATP production (PubMed:29478834)