FUCA1 anticorps (N-Term)
Aperçu rapide pour FUCA1 anticorps (N-Term) (ABIN2785639)
Antigène
Voir toutes FUCA1 AnticorpsReactivité
Hôte
Clonalité
Conjugué
Application
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Épitope
- N-Term
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Séquence
- PSPVSWNWNS KDVGPHRDLV GELGTALRKR NIRYGLYHSL LEWFHPLYLL
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Homologie
- Cow: 100%, Dog: 93%, Guinea Pig: 93%, Horse: 93%, Human: 100%, Mouse: 100%, Pig: 100%, Rabbit: 79%, Rat: 100%
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Attributs du produit
- This is a rabbit polyclonal antibody against FUCA1. It was validated on Western Blot using a cell lysate as a positive control.
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Purification
- Affinity Purified
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Immunogène
- The immunogen is a synthetic peptide directed towards the N terminal region of human FUCA1
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Indications d'application
- Optimal working dilutions should be determined experimentally by the investigator.
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Commentaires
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Antigen size: 466 AA
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Restrictions
- For Research Use only
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Format
- Liquid
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Concentration
- Lot specific
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Buffer
- Liquid. Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.
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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 freeze-thaw cycles.
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Stock
- -20 °C
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Stockage commentaire
- For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.
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- FUCA1 (Fucosidase, alpha-L- 1, Tissue (FUCA1))
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Autre désignation
- FUCA1
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Sujet
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Alpha-L-fucosidase (EC 3.2.1.51) is a lysosomal enzyme involved in the degradation of fucose-containing glycoproteins and glycolipids. At least 2 separate polymorphic alpha-L-fucosidases are recognized in man: that in tissues, FUCA1, which is deficient in fucosidosis, and that in plasma, FUCA2. Fucosidosis is an autosomal recessive lysosomal storage disease caused by defective alpha-L-fucosidase with accumulation of fucose in the tissues. Different phenotypes include clinical features such as neurologic deterioration, growth retardation, visceromegaly, and seizures in a severe early form, coarse facial features, angiokeratoma corporis diffusum, spasticity and delayed psychomotor development in a longer surviving form, and an unusual spondylometaphyseoepiphyseal dysplasia in yet another form.[supplied by OMIM]. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications.
Alias Symbols: FUCA
Protein Interaction Partner: PAXIP1, BARD1, APP, CUL3, VAV2, USP21, MARK2,
Protein Size: 466 -
Poids moléculaire
- 54 kDa
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ID gène
- 2517
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NCBI Accession
- NM_000147, NP_000138
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UniProt
- P04066
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Pathways
- Glycosaminoglycan Metabolic Process
Antigène
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