P5CS anticorps (C-Term)
Aperçu rapide pour P5CS anticorps (C-Term) (ABIN569586)
Antigène
Voir toutes P5CS (ALDH18A1) AnticorpsReactivité
Hôte
Clonalité
Conjugué
Application
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Épitope
- C-Term
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Séquence
- C-SEHGSLKYLH
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Specificité
- This antibody is expected to recognize both reported isoforms of ALDH18A1 (NP_002851.2, NP_001017423.1).
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Réactivité croisée (Details)
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Species reactivity (expected):Rat, Canine (Dog), Cow, Pig.
Species reactivity (tested):Human, Mouse. -
Purification
- Ammonium Sulphate Precipitation followed by Antigen Affinity Chromatography using the immunizing peptide.
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Immunogène
- Peptide with sequence from the C Terminus of the protein sequence according to NP_002851.2, NP_001017423.1. Genename: ALDH18A1
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Indications d'application
- Optimal working dilution should be determined by the investigator.
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Restrictions
- For Research Use only
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Concentration
- 0.5 mg/mL
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Buffer
- Tris saline, pH 7.3 containing 0.02 % Sodium Azide as preservative and 0.5 % BSA as stabilizer.
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Agent conservateur
- Sodium azide
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Précaution d'utilisation
- This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Conseil sur la manipulation
- Avoid repeated freezing and thawing.
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Stock
- 4 °C/-20 °C
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Stockage commentaire
- Store the antibody undiluted at 2-8 °C for one month or (in aliquots) at -20 °C for longer.
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- P5CS (ALDH18A1) (Aldehyde Dehydrogenase 18 Family, Member A1 (ALDH18A1))
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Autre désignation
- ALDH18A1
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Sujet
- Defects in ALDH18A1 are the cause of mental retardation-joint hypermobility-skin laxity with or without metabolic abnormalities (MRJHSL) [MIM:612652]. Clinical manifestations include microcephaly, progressive neurologic dysfunction, mental retardation, progeroid appearance, joint hypermobility, skin laxity and hyperelasticity, cataracts. Some patients manifest metabolic disturbances such as hyperammonemia, hypoornithinemia, hypocitrullinemia, hypoargininemia and hypoprolinemia.Synonyms: Aldehyde dehydrogenase family 18 member A1, Delta-1-pyrroline-5-carboxylate synthetase, GSAS, P5CS, PYCS
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Poids moléculaire
- 87.3kDa (NP_002851.2).
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ID gène
- 5832
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NCBI Accession
- NP_001017423
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UniProt
- P54886
Antigène
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