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OPA1 Proteine (OPA1)

Bezeichnung:
Optic Atrophy 1 (Autosomal Dominant) Proteine (OPA1)
Auf www.antikoerper-online.de finden Sie aktuell 9 Optic Atrophy 1 (Autosomal Dominant) (OPA1) Proteine von 3 unterschiedlichen Herstellern. Zusätzlich bieten wir Ihnen OPA1 Antikörper (63) und viele weitere Produktgruppen zu diesem Protein an. Insgesamt sind aktuell 75 OPA1 Produkte verfügbar.
Synonyme:
1200011N24Rik, AI225888, AI847218, fk62d06, largeG, lilr3, MGM1, mKIAA0567, NPG, NTG, wu:fb77a10, wu:fk62d06, zgc:92092
alle Proteine anzeigen Gen GeneID UniProt
OPA1 74143 P58281
Ratte OPA1 OPA1 171116 Q2TA68
OPA1 4976 O60313

Weitere Synonyme anzeigen

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Weitere Proteine zu OPA1 Interaktionspartnern

Mouse (Murine) Optic Atrophy 1 (Autosomal Dominant) (OPA1) Interaktionspartner

  1. OPA1 modulates cristae morphology but is dispensable for cristae junction formation. Endogenous OPA1 and MIC60 show a physical interaction.

  2. Opa1 deficiency was associated with increased sensitivity to Ischemia-Reperfusion Injuries, imbalance in dynamic mitochondrial Ca2 (zeige CA2 Proteine)+ uptake, and subsequent increase in NCX (zeige SLC8A1 Proteine) activity.

  3. Whereas Parkin (zeige PARK2 Proteine) has been reported to positively regulate the expression of OPA1 through NEMO (zeige IKBKG Proteine), herein we found that PARK2 (zeige PARK2 Proteine) overexpression did not modify the expression of OPA1.

  4. stress-induced OMA1 (zeige OMA1 Proteine) activation and guanosine triphosphatase OPA1 cleavage limit mitochondrial fusion and promote neuronal death

  5. Data suggest that in a mouse model of neonatal hypoxic-ischemic brain injury, the expression of mitochondrial shaping proteins, such as OPA1 and Yme1L (zeige YME1L1 Proteine), are altered; in vitro and in vivo, OPA1 is cleaved to shorter forms and Yme1L (zeige YME1L1 Proteine) expression is reduced.

  6. results indicate that the OPA1-dependent cristae remodeling pathway is a fundamental, targetable determinant of tissue damage in vivo.

  7. cristae shape amelioration by controlled Opa1 overexpression improves two mouse models of mitochondrial disease.

  8. unprocessed OPA1 is sufficient to maintain heart function, OMA1 (zeige OMA1 Proteine) is a critical regulator of cardiomyocyte survival, and mitochondrial morphology and cardiac metabolism are intimately linked.

  9. Data suggest that mitochondrial fusion and fission events are regulated by four GTPases: OPA1, Drp1 (dynamin 1-like protein (zeige DNM1L Proteine)), and Mfn1 (zeige MFN1 Proteine)/2 (mitofusin 1 (zeige MFN1 Proteine) and 2). [REVIEW]

  10. Photoresponsive RGCs are protected against cell death due to the Opa1 mutation, but not by melanopsin (zeige OPN4 Proteine) expression itself.

Human Optic Atrophy 1 (Autosomal Dominant) (OPA1) Interaktionspartner

  1. The gene signature of OPA1, CTSA (zeige CTSA Proteine), NDUFA1 (zeige NDUFA1 Proteine), STK10 (zeige STK10 Proteine) and PRDX1 (zeige PRDX1 Proteine) was able to identify patients post-implant with a sensitivity of 91% and a specificity of 86% in discrimination between post-implant group and healthy controls.

  2. The architecture of dendritic arborization in patients with OPA1 mutations is not known, but our data support the idea that loss of dendritic arborization may be involved in the pathogenesis of DOA rather than just population loss.

  3. OPA1 disclosed a de novo heterozygous deletion c.2012+4_2012+7delAGTA resulting in exon 18 and 19 skipping, which was not detected in healthy family members.

  4. This study demonstrated increased mitophagy and excessive mitochondrial fragmentation in primary human cultures associated with DOA plus due to biallelic OPA1 mutations.

  5. The present study identified novel compound heterozygous OPA1 mutations in a patient with recessive optic atrophy, sensorimotor neuropathy and congenital cataracts, indicating an expansion of the clinical spectrum of pathologies associated with OPA1 mutations.

  6. Optic atrophy type 1, caused by mutations in the OPA1 gene is believed to be the most common hereditary optic neuropathy, and most patients inherit a mutation from an affected parent. In this study we used whole-exome sequencing to investigate the genetic aetiology in a patient affected with isolated optic atrophy. Exome results identified a novel de novo OPA1 mutation.

  7. Findings show a new mode of regulation of the mitochondrial fusion proteins, Mfns degradation or OPA1 processing, in response to mitochondrial morphology.

  8. Loss of OPA1 protein function by pathogenic OPA1 gene mutation induces increased mitochondrial fragmentation that promotes instability of the mitochondrial respiratory chain complexes.

  9. Two heterozygous mutations, p.T414P (c.1240A>C) and p.T540P (c.1618A>C), located in the GTPase (zeige RACGAP1 Proteine) and middle domains of OPA1, respectively, were identified in two patients.These two different conformational changes might result in decreased GTPase (zeige RACGAP1 Proteine) activities that trigger autosomal dominant optic atrophy associated with auditory neuropathy spectrum disorder

  10. A causal link between a pathogenic homozygous OPA1 mutation and hypertrophic cardiomyopathy with optic atrophy was established.It emphasise the vital role played by OPA1 in mitochondrial biogenesis and mtDNA maintenance.

Zebrafish Optic Atrophy 1 (Autosomal Dominant) (OPA1) Interaktionspartner

  1. Opa1 is required for proper mitochondrial metabolism in early development

OPA1 Protein Überblick

Protein Überblick

This gene product is a nuclear-encoded mitochondrial protein with similarity to dynamin-related GTPases. It is a component of the mitochondrial network. Mutations in this gene have been associated with optic atrophy type 1, which is a dominantly inherited optic neuropathy resulting in progressive loss of visual acuity, leading in many cases to legal blindness. Multiple transcript variants encoding different isoforms have been found for this gene.

Alternative names and synonyms associated with OPA1

  • optic atrophy 1 (Opa1)
  • optic atrophy 1 (autosomal dominant) (OPA1)
  • optic atrophy 1 (human) (opa1)
  • 1200011N24Rik Protein
  • AI225888 Protein
  • AI847218 Protein
  • fk62d06 Protein
  • largeG Protein
  • lilr3 Protein
  • MGM1 Protein
  • mKIAA0567 Protein
  • NPG Protein
  • NTG Protein
  • wu:fb77a10 Protein
  • wu:fk62d06 Protein
  • zgc:92092 Protein

Bezeichner auf Proteinebene für OPA1

dynamin-like 120 kDa protein, mitochondrial , large GTP-binding protein , largeG , optic atrophy 1 homolog , optic atrophy protein 1 homolog , RN protein , optic atrophy 1 (autosomal dominant) , optic atrophy 1-like protein , dynamin-like guanosine triphosphatase , mitochondrial dynamin-like GTPase , optic atrophy protein 1

GENE ID SPEZIES
74143 Mus musculus
171116 Rattus norvegicus
4976 Homo sapiens
424900 Gallus gallus
477129 Canis lupus familiaris
524142 Bos taurus
100172619 Pongo abelii
492332 Danio rerio
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