Mannosyl-Oligosaccharide Glucosidase (MOGS) (C-Term) Antikörper

Details zu Produkt Nr. ABIN357670, Anbieter: Anmelden zum Anzeigen
Antigen
  • Afu6g04210
  • AO090701000141
  • Mogs
  • CDG2B
  • CWH41
  • DER7
  • GCS1
  • 1810017N02Rik
  • AI181835
  • Gcs1
  • gcs1
  • im:7160827
  • wu:fe50a12
  • wu:fk09a10
  • zgc:158312
  • mannosyl-oligosaccharide glucosidase
  • Mannosyl oligosaccharide glucosidase
  • mannosyl oligosaccharide glucosidase
  • glucosidase 1
  • AFUA_6G04210
  • Tc00.1047053511015.10
  • Tc00.1047053511805.10
  • AaeL_AAEL012479
  • AOR_1_260114
  • MGYG_00305
  • TERG_01248
  • mogs
  • TTHERM_00636930
  • LOAG_03690
  • Gcs1
  • MOGS
  • Mogs
Epitop
C-Term
22
16
4
3
2
1
Reaktivität
Human, Maus
68
35
21
2
2
2
2
2
2
1
1
1
Wirt
Kaninchen
68
Klonalität
Polyklonal
Konjugat
Unkonjugiert
5
5
5
4
4
4
1
1
1
1
1
1
1
1
1
1
1
Applikation
Enzyme Immunoassay (EIA), Western Blotting (WB)
53
32
13
5
2
1
1
1
1
Optionen
Hersteller
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Hersteller Produkt- Nr.
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Immunogen This antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide selected form the C-terminal region of human GCS1.
Isotyp Ig
Spezifität This antibody is specific to GCS1 (C-term).
Reinigung Protein G Chromatography, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS.
Andere Bezeichnung MOGS / GCS1 (MOGS Antibody Abstract)
Hintergrund GCS1 cleaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner. Defects in GCS1 are the cause of type IIb congenital disorder of glycosylation (CDGIIb). This syndrome is also known as glucosidase I deficiency and is characterized by marked generalized hypotonia and hypomotility of the neonate, dysmorphic features, including a prominent occiput, short palpebral fissures, retrognathia, high arched palate, generalized edema, and hypoplastic genitalia. Symptoms include hepatomegaly, hypoventilation, feeding problems and seizures. The clinical course is progressive and survival is at most a few months.Synonyms: Mannosyl-oligosaccharide glucosidase, Processing A-glucosidase I
Molekulargewicht 91840 Da
Gen-ID 7841, 5874
UniProt Q13724
Applikationshinweise ELISA: 1/1,000. Western Blot: 1/100-1/500.
Other applications not tested.
Optimal dilutions are dependent on conditions and should be determined by the user.
Beschränkungen Nur für Forschungszwecke einsetzbar
Format Liquid
Konzentration 0.25 mg/mL
Buffer PBS with 0.09 % (W/V) Sodium Azide as preservative.
Konservierungsmittel Sodium azide
Vorsichtsmaßnahmen This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Handhabung Avoid repeated freezing and thawing.
Lagerung 4 °C/-20 °C
Informationen zur Lagerung Store the antibody undiluted at 2-8 °C for one month or (in aliquots) at-20 °C for longer.
Bilder des Herstellers
 image for anti-Mannosyl-Oligosaccharide Glucosidase (MOGS) (C-Term) antibody (ABIN357670) anti-Mannosyl-Oligosaccharide Glucosidase (MOGS) (C-Term) antibody
Allgemeine Veröffentlichungen Völker, De Praeter, Hardt, Breuer, Kalz-Füller, Van Coster, Bause: "Processing of N-linked carbohydrate chains in a patient with glucosidase I deficiency (CDG type IIb)." in: Glycobiology, Vol. 12, Issue 8, pp. 473-83, 2002 (PubMed).

De Praeter CM, Gerwig, Bause, Nuytinck, Vliegenthart, Breuer, Kamerling, Espeel, Martin, De Paepe AM, Chan, Dacremont, Van Coster RN: "A novel disorder caused by defective biosynthesis of N-linked oligosaccharides due to glucosidase I deficiency." in: American journal of human genetics, Vol. 66, Issue 6, pp. 1744-56, 2001 (PubMed).

Kalz-Füller, Bieberich, Bause: "Cloning and expression of glucosidase I from human hippocampus." in: European journal of biochemistry / FEBS, Vol. 231, Issue 2, pp. 344-51, 1995 (PubMed).

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