Human, Mouse, Rat TBX1 Polyclonal Antibody - 100 ul (1ug/ul)

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2775,36 zł 2775.36 PLN 2775,36 zł

588,00 €

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    Terms and Conditions
    Gwarantowany zwrot pieniędzy przez 30dni
    Wysyłka w ciągu 2-3 dni roboczych

    Applications: WB, ELISA, FCM


    Reactivity: Human, Mouse, Rat


    Predicted reactivity: Dog, Cow, Pig, Horse, Chicken

     


       


    OVERVIEW
    CATALOG #bs-8257R
    PRODUCT NAMETBX1 Polyclonal Antibody
    APPLICATIONSWB, ELISA, FCM
    REACTIVITYHuman, Mouse, Rat
    PREDICTED REACTIVITYDog, Cow, Pig, Horse, Chicken
    SPECIFICATIONS
    CONJUGATIONUnconjugated
    HOSTRabbit
    SOURCEKLH conjugated synthetic peptide derived from human TBX1
    IMMUNOGEN RANGE165-270/398
    CLONALITYPolyclonal
    ISOTYPEIgG
    CONCENTRATION1ug/ul
    PURIFICATIONPurified by Protein A.
    STORAGE BUFFER0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
    STORAGE CONDITIONShipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
    TARGET
    GENE ID6899
    SUBCELLULAR LOCATIONNucleus
    SYNONYMSBrachyury; CAFS; CTHM; DGCR; DGS; DORV; T box 1; T box 1 transcription factor; T box 1 transcription factor C; T box; T box protein 1; T box transcription factor TBX 1; T box transcription factor TBX1; T-box 1; T-box protein 1; T-box transcription factor TBX1; TBX 1; TBX 1C; tbx1; TBX1_HUMAN; TBX1C; Testis specic T box protein; Testis-specic T-box protein; TGA; VCFS.
    BACKGROUNDProbable transcriptional regulator involved in developmental processes. Is required for normal development of the pharyngeal arch arteries.Involvement in disease:Haploinsufficiency of the TBX1 gene is responsible for most of the physical malformations present in DiGeorge syndrome (DGS) and velocardiofacial syndrome (VCFS) . DGS is characterized by the association of several malformations: hypoplastic thymus and parathyroid glands, congenital conotruncal cardiopathy, and a subtle but characteristic facial dysmorphology. VCFS is marked by the association of congenital conotruncal heart defects, cleft palate or velar insufficiency, facial dysmorpholgy and learning difficulties. It is now accepted that these two syndromes represent two forms of clinical expression of the same entity manifesting at different stages of life.Defects in TBX1 are a cause of DiGeorge syndrome (DGS) .Defects in TBX1 are a cause of velocardiofacial syndrome (VCFS) .Defects in TBX1 are a cause of conotruncal heart malformations (CTHM). CTHM consist of cardiac outflow tract defects, such as tetralogy of Fallot, pulmonary atresia, double-outlet right ventricle, truncus arteriosus communis, and aortic arch anomalies.
    APPLICATION DILUTION
    WB1:300-5000
    ELISA1:500-1000
    FCM1:20-100


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