HMBS anticorps (N-Term)
Aperçu rapide pour HMBS anticorps (N-Term) (ABIN2785718)
Antigène
Voir toutes HMBS AnticorpsReactivité
Hôte
Clonalité
Conjugué
Application
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Épitope
- N-Term
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Fonction
- HMBS Antibody - N-terminal region
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Séquence
- MRVIRVGTRK SQLARIQTDS VVATLKASYP GLQFEIIAMS TTGDKILDTA
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Homologie
- Cow: 100%, Dog: 100%, Guinea Pig: 100%, Horse: 100%, Human: 100%, Mouse: 100%, Rabbit: 100%, Rat: 100%, Sheep: 100%, Zebrafish: 100%
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Attributs du produit
- This is a rabbit polyclonal antibody against HMBS. 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 HMBS
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Indications d'application
- Optimal working dilution should be determined by the investigator.
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Commentaires
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We also sell a specific blocking peptide that can be used in combination with this antibody. You can find the blocking peptide under AAP54451-100UG
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Restrictions
- For Research Use only
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Format
- Liquid
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Concentration
- 0.5 mg/mL
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Buffer
- 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
- prevent freeze-thaw cycles
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Stock
- 4 °C,-20 °C
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Stockage commentaire
- For short term use, store at 2-8C up to 1 week. For long term storage, store at -20 °C in small aliquots to prevent freeze-thaw cycles.
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: "Acute intermittent porphyria with peripheral neuropathy: a follow-up study after hematin treatment." dans: Journal of the neurological sciences, Vol. 260, Issue 1-2, pp. 231-5, (2007) (PubMed).
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- HMBS (Hydroxymethylbilane Synthase (HMBS))
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Autre désignation
- HMBS
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Sujet
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Background Information: HMBS is a member of the hydroxymethylbilane synthase superfamily. It is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria.This gene encodes a member of the hydroxymethylbilane synthase superfamily. The encoded protein is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria. Alternatively spliced transcript variants encoding different isoforms have been described.
Gene Name: Hydroxymethylbilane synthase
Alternative Symbols: UPS, PBGD, PORC, PBG-D
Protein Name: Porphobilinogen deaminase
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Poids moléculaire
- 38kDa
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ID gène
- 3145
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NCBI Accession
- NP_001019553
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UniProt
- P08397
Antigène
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