SLC19A3 anticorps (C-Term)
Aperçu rapide pour SLC19A3 anticorps (C-Term) (ABIN5516361)
Antigène
Voir toutes SLC19A3 (Slc19a3) AnticorpsReactivité
Hôte
Clonalité
Conjugué
Application
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Épitope
- C-Term
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Fonction
- SLC19A3 Antibody - C-terminal region
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Séquence
- VYGSYFAVIA GIFLMRSMYI TYSTKSQKDV QSPAPSENPD VSHPEEESNI
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Purification
- Affinity purified
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Immunogène
- The immunogen is a synthetic peptide directed towards the C terminal region of human SLC19A3
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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 AAP74823-100UG
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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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- SLC19A3 (Slc19a3) (Solute Carrier Family 19, Member 3 (Slc19a3))
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Autre désignation
- SLC19A3
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Sujet
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Background Information: This gene encodes a ubiquitously expressed transmembrane thiamine transporter that lacks folate transport activity. Mutations in this gene cause biotin-responsive basal ganglia disease (BBGD); a recessive disorder manifested in childhood that progresses to chronic encephalopathy, dystonia, quadriparesis, and death if untreated. Patients with BBGD have bilateral necrosis in the head of the caudate nucleus and in the putamen. Administration of high doses of biotin in the early progression of the disorder eliminates pathological symptoms while delayed treatment results in residual paraparesis, mild mental retardation, or dystonia. Administration of thiamine is ineffective in the treatment of this disorder. Experiments have failed to show that this protein can transport biotin. Mutations in this gene also cause a Wernicke's-like encephalopathy.
Gene Name: solute carrier family 19 member 3
Alternative Symbols: BBGD, THMD2, THTR2, thTr-2
Protein Name: thiamine transporter 2
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Poids moléculaire
- 55 kDa
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ID gène
- 80704
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NCBI Accession
- NP_079519
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UniProt
- Q9BZV2
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Pathways
- Dicarboxylic Acid Transport
Antigène
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