Optic Atrophy 3 (Autosomal Recessive, with Chorea and Spastic Paraplegia) Proteine (OPA3)

The mouse ortholog of this protein co-purifies with the mitochondrial inner membrane. Zusätzlich bieten wir Ihnen OPA3 Antikörper (25) und viele weitere Produktgruppen zu diesem Protein an.

alle Proteine anzeigen Gen GeneID UniProt
OPA3 403187 Q505D7
OPA3 80207 Q9H6K4
OPA3 308409  
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Top OPA3 Proteine auf antikoerper-online.de

Showing 9 out of 10 products:

Katalog Nr. Origin Quelle Konjugat Bilder Menge Anbieter Lieferzeit Preis Details
HOST_Escherichia coli (E. coli) Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Anmelden zum Anzeigen 26 bis 31 Tage
4.115,41 €
Details
Insektenzellen Maus His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Anmelden zum Anzeigen 46 Days
5.173,33 €
Details
Hefe Zebrafisch His tag   1 mg Anmelden zum Anzeigen 58 bis 70 Tage
2.240,16 €
Details
Hefe Rind (Kuh) His tag   1 mg Anmelden zum Anzeigen 58 bis 70 Tage
2.341,43 €
Details
HOST_HEK-293 Cells Human Myc-DYKDDDDK Tag Validation with Western Blot 20 μg Anmelden zum Anzeigen 6 bis 8 Tage
748,00 €
Details
HOST_Wheat germ Human GST tag 10 μg Anmelden zum Anzeigen 7 bis 8 Tage
345,60 €
Details
HOST_Escherichia coli (E. coli) Human T7 tag,His tag   100 μg Anmelden zum Anzeigen 13 bis 17 Tage
645,71 €
Details
HOST_Escherichia coli (E. coli) Maus T7 tag,His tag   100 μg Anmelden zum Anzeigen 13 bis 17 Tage
691,43 €
Details
HOST_Escherichia coli (E. coli) Ratte T7 tag,His tag   100 μg Anmelden zum Anzeigen 13 bis 17 Tage
706,67 €
Details

OPA3 Proteine nach Spezies und Herkunft

Origin Exprimiert in Konjugat
Mouse (Murine) ,
,
Human , ,
, , ,
Rat (Rattus)

Weitere Proteine zu Optic Atrophy 3 (Autosomal Recessive, with Chorea and Spastic Paraplegia) (OPA3) Interaktionspartnern

Zebrafish Optic Atrophy 3 (Autosomal Recessive, with Chorea and Spastic Paraplegia) (OPA3) Interaktionspartner

  1. mitochondrial OPA3 is required to limit HMG-CoA-derived MGC and protect the electron transport chain against inhibitory compounds in a zebrafish model of Costeff Syndrome

Mouse (Murine) Optic Atrophy 3 (Autosomal Recessive, with Chorea and Spastic Paraplegia) (OPA3) Interaktionspartner

  1. Opa3(L122P) mice displayed craniofacial abnormalities, including undergrowth of the lower mandible, accompanied in some individuals by cranial asymmetry and incisor malocclusion

  2. Opa3, a novel regulator of mitochondrial function, controls thermogenesis and abdominal fat mass in a mouse model for Costeff syndrome.

  3. Mutant Opa3 protein retains its mitochondrial localization and induces disrupted mitochondrial morphology. Opa3 accumulates in the lens. The results may reflect a slow turnover of Opa3 protein in vivo and may be important in normal lens physiology.

  4. to investigate the OPA3 function we have generated a novel ENU-induced mutant mouse carrying a missense mutation in the OPA3 gene. In the heterozygous state, the mice appear uncompromised. In the homozygous state mice display some of the features of MGA.

Cow (Bovine) Optic Atrophy 3 (Autosomal Recessive, with Chorea and Spastic Paraplegia) (OPA3) Interaktionspartner

  1. The present study is the first report of the OPA3 mutation in dilated cardiomyopathy affected cows outside Switzerland.

  2. the nonsense mutation c.343C>T in the bovine OPA3 gene causes the late-onset dilated cardiomyopathy in Red Holstein cattle.

Human Optic Atrophy 3 (Autosomal Recessive, with Chorea and Spastic Paraplegia) (OPA3) Interaktionspartner

  1. Mutations of the OPA3 gene can cause either autosomal dominant or autosomal recessive optic atrophy.

  2. Report the results of a comprehensive study on OPA3 mutations, including the mutation spectrum and its prevalence in a large cohort of ADOA patients, the associated clinical phenotype and the functional characterisation of a newly identified OPA3 mutant.

  3. A novel missense mutation identifies OPA3 as the cause of a complex neurological disorder with marked lower limb dystonia.

  4. OPA1 mutations are the most common genetic defects identified in patients with suspected Autosomal-dominant optic atrophy (DOA), whereas OPA3 mutations are very rare in isolated optic atrophy cases.

  5. OPA3, as an integral mitochondrial outer membane perotein, has a crucial role in mitochondrial fission, and provides a direct link between mitochondrial morphology and optic atrophy.

  6. two missense mutations in OPA3 in two families affected by autosomal dominant optic atrophy and cataract (ADOAC)

  7. OPA1 mutations, mtDNA mutations, and OPA3 mutations in 980 patients Leber's hereditary optic neuropathy and autosomal dominant optic atrophy

  8. The mouse ortholog of OPA3 purifies with mitochondrial inner membranes.

  9. The mouse ortholog of OPA3 purifies with mitochondria

  10. type III 3-methylglutaconic aciduria (optic atrophy plus syndrome, or Costeff optic atrophy syndrome): identification of the OPA3 gene and its founder mutation in Iraqi Jews

OPA3 Protein Überblick

Protein Überblick

The mouse ortholog of this protein co-purifies with the mitochondrial inner membrane. Mutations in this gene have been shown to result in 3-methylglutaconic aciduria type III and autosomal dominant optic atrophy and cataract. Multiple transcript variants encoding different isoforms have been found for this gene.

Genbezeichner und Symbole assoziert mit OPA3

  • optic atrophy 3 (opa3)
  • optic atrophy 3 (Opa3)
  • optic atrophy 3 (autosomal recessive, with chorea and spastic paraplegia) (OPA3)
  • D630048P19Rik Protein
  • Gm472 Protein
  • Gm1425 Protein
  • MGA3 Protein

Bezeichner auf Proteinebene für OPA3

optic atrophy 3 protein homolog , mitochondrial optic atrophy 3 protein , Optic atrophy 3 (Iraqi-Jewish 'optic atrophy plus') , optic atrophy 3 protein , optic atrophy 3 (autosomal recessive, with chorea and spastic paraplegia)

GENE ID SPEZIES
497278 Danio rerio
403187 Mus musculus
523579 Bos taurus
80207 Homo sapiens
308409 Rattus norvegicus
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