Opa1 - OPA1, mitochondrial dynamin like GTPase Gene

Also Known as lilr3; mKIAA0567; 1200011N24Rik

Species: Mus musculus

Gene Type: protein coding

Gene ID: 74143

Summary

Predicted to enable several functions, including GTPase activity; anion binding activity; and microtubule binding activity. Involved in negative regulation of endoplasmic reticulum stress-induced intrinsic apoptotic signaling pathway and negative regulation of release of cytochrome c from mitochondria. Acts upstream of or within mitochondrial fusion and neural tube closure. Located in dendrite; mitochondrial inner membrane; and mitochondrial intermembrane space. Is expressed in several structures, including heart; liver; lung; skin; and visual system. Used to study optic atrophy. Human ortholog(s) of this gene implicated in Behr syndrome; dominant optic atrophy plus syndrome; mitochondrial DNA depletion syndrome 14; optic atrophy; and optic atrophy 1. Orthologous to human OPA1 (OPA1 mitochondrial Dynamin like GTPase). [provided by Alliance of Genome Resources, Apr 2022]

Opa1 Products (6)

mRNA	Protein	Name
NM_001199177.2	NP_001186106.1	dynamin-like 120 kDa protein, mitochondrial isoform 1
NM_001403170.1	NP_001390099.1	dynamin-like 120 kDa protein, mitochondrial isoform 3
NM_001403171.1	NP_001390100.1	dynamin-like 120 kDa protein, mitochondrial isoform 4
NM_001403172.1	NP_001390101.1	dynamin-like 120 kDa protein, mitochondrial isoform 4
NM_001403182.1	NP_001390111.1	dynamin-like 120 kDa protein, mitochondrial isoform 2
NM_133752.4	NP_598513.1	dynamin-like 120 kDa protein, mitochondrial isoform 2

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	References	Source
enables GTPase-dependent fusogenic activity	IDA IDA: Inferred from direct assay	28636943	MGI
enables protein binding	IPI IPI: Inferred from physical interaction	21081504	MGI

Biological Process GO Annotation	Evidence	References	Source
involved in cristae formation	IDA IDA: Inferred from direct assay	25298396	MGI
involved in cristae formation	IMP IMP: Inferred from mutant phenotype	24055366	MGI
involved in inner mitochondrial membrane organization	IDA IDA: Inferred from direct assay	28636943	MGI
involved in inner mitochondrial membrane organization	IMP IMP: Inferred from mutant phenotype	36171294	MGI
involved in mitochondrial fusion	IMP IMP: Inferred from mutant phenotype	15899901	MGI
involved in mitochondrial inner membrane fusion	IDA IDA: Inferred from direct assay	24703695	MGI
involved in negative regulation of endoplasmic reticulum stress-induced intrinsic apoptotic signaling pathway	IMP IMP: Inferred from mutant phenotype	23453807	MGI
involved in negative regulation of intrinsic apoptotic signaling pathway	IMP IMP: Inferred from mutant phenotype	16839884	MGI
involved in negative regulation of release of cytochrome c from mitochondria	IMP IMP: Inferred from mutant phenotype	16839884	MGI
acts upstream of or within neural tube closure	IMP IMP: Inferred from mutant phenotype	15755804	MGI
involved in positive regulation of T-helper 17 cell lineage commitment	IMP IMP: Inferred from mutant phenotype	36171294	MGI
involved in positive regulation of interleukin-17 production	IMP IMP: Inferred from mutant phenotype	36171294	MGI

Cellular Component GO Annotation	Evidence	References	Source
located in dendrite	IDA IDA: Inferred from direct assay	11847212	MGI
is active in mitochondrial inner membrane	IDA IDA: Inferred from direct assay	38225406	MGI
located in mitochondrial inner membrane	IDA IDA: Inferred from direct assay	28746876	MGI
located in mitochondrial inner membrane	IMP IMP: Inferred from mutant phenotype	16839884	MGI
is active in mitochondrial intermembrane space	IDA IDA: Inferred from direct assay	38225406	MGI
located in mitochondrial intermembrane space	IDA IDA: Inferred from direct assay	11847212	MGI
located in mitochondrion	IDA IDA: Inferred from direct assay	11847212	MGI

EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

Protein Preferred Names	Protein Names
dynamin-like 120 kDa protein, mitochondrial	large GTP-binding protein largeG optic atrophy 1 homolog optic atrophy protein 1 homolog

Related Agents

Pre-clinical Phase

Pre-clinical Phase

Bioactive Molecules (14)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-126124	AP39		96.35%	Unkown
HY-139109	IR-783		99.87%	Unkown
HY-177306	Opitor-0	Inhibitor	98.02%	Unkown
HY-158162	SIRT3 activator 1	Activator	98.14%	Unkown
HY-116364B	AZT triphosphate tetraammonium		99.25%	Unkown
HY-RS16968	Opa1 Mouse Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-116364	AZT triphosphate		/	Unkown
HY-116364A	AZT triphosphate TEA		/	Unkown
HY-RS08281	MED12 Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS09789	OPA1 Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-131037	MB-0223	Inhibitor	/	Unkown
HY-180786	DdBIC		/	Unkown
HY-RS18290	Med12 Mouse Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS23411	Opa1 Rat Pre-designed siRNA Set A	Pre-designed Set	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Homo sapiens	Opa1	NCBI	NCBI:4976