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    MCT 8 Rabbit pAb (bs-11434R)  
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    產(chǎn)品編號 bs-11434R
    英文名稱(chēng) MCT 8 Rabbit pAb
    中文名稱(chēng) 甲狀腺激素轉運蛋白/單羧酸轉運蛋白7/8抗體
    別    名 SLC16A2; AHDS; DXS 128; DXS 128E; DXS128; DXS128 E; DXS128E; XPCT; MCT 7; MCT7; MCT8; Monocarboxylate transporter 7; Monocarboxylate transporter 8; MOT8_HUMAN; MRX 22; MRX22; SLC16 A2; SLC16A 2; Solute carrier family 16(monocarboxylic acid transporters), member 2; Solute carrier family 16 member 2; Solute carrier family 16, member 2(monocarboxylic acid transporter 8); Solute carrier family 16, member 2(thyroid hormone transporter); Solute carrier family 16, member 2; X linked PEST containing transporter; X-linked PEST-containing transporter.  
    研究領(lǐng)域 細胞生物  神經(jīng)生物學(xué)  信號轉導  生長(cháng)因子和激素  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    克 隆 號
    交叉反應 Human,Mouse,Rat (predicted: Rabbit)
    產(chǎn)品應用 WB=1:500-2000,ELISA=1:5000-10000
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    理論分子量 59 kDa
    檢測分子量
    細胞定位 細胞膜 
    性    狀 Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from mouse MOT8/SLC16A2 <Extracellular>
    亞    型 IgG
    純化方法 affinity purified by Protein A
    緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
    保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
    注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
    PubMed PubMed
    產(chǎn)品介紹 This gene encodes an integral membrane protein that functions as a transporter of thyroid hormone. The encoded protein facilitates the cellular importation of thyroxine (T4), triiodothyronine (T3), reverse triiodothyronine (rT3) and diidothyronine (T2). This gene is expressed in many tissues and likely plays an important role in the development of the central nervous system. Loss of function mutations in this gene are associated with psychomotor retardation in males while females exhibit no neurological defects and more moderate thyroid-deficient phenotypes. This gene is subject to X-chromosome inactivation. Mutations in this gene are the cause of Allan-Herndon-Dudley syndrome. [provided by RefSeq, Mar 2012]

    Function:
    Very active and specific thyroid hormone transporter. Stimulates cellular uptake of thyroxine (T4), triiodothyronine (T3), reverse triiodothyronine (rT3) and diidothyronine. Does not transport Leu, Phe, Trp or Tyr.

    Subunit:
    Homodimer.

    Subcellular Location:
    Cell membrane; Multi-pass membrane protein

    Tissue Specificity:
    Highly expressed in liver and heart.

    DISEASE:
    Defects in SLC16A2 are the cause of monocarboxylate transporter 8 deficiency (MCT8 deficiency) [MIM:300523]; also known as Allan-Herndon-Dudley syndrome (AHDS). MCT8 deficiency consists of a severe form of X-linked psychomotor retardation combined with abnormal thyroid hormone (TH) levels. Thyroid hormone deficiency can be caused by defects of hormone synthesis and action, but it has also been linked to a defect in cellular hormone transport. Affected patients are males with abnormal relative concentrations of three circulating iodothyronines, as well as severe neurological abnormalities, including global developmental delay, central hypotonia, spastic quadriplegia, dystonic movements, rotary nystagmus, and impaired gaze and hearing. Heterozygous females had a milder thyroid phenotype and no neurological defects.

    Similarity:
    Belongs to the major facilitator superfamily. Monocarboxylate porter (TC 2.A.1.13) family.

    Database links:

    Entrez Gene: 6567 Human

    Omim: 300095 Human

    SwissProt: P36021 Human

    Unigene: 75317 Human



    產(chǎn)品圖片
    Sample: Lane 1: Human U251 cell lysates Lane 2: Human U87MG cell lysates Lane 3: Human SH-SY5Y cell lysates Primary: Anti-MCT 8 (bs-11434R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 59 kDa Observed band size: 50 kDa
    Sample: Heart (Mouse) Lysate at 40 ug Primary: Anti- MOT8 (bs-11434R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 59 kD Observed band size: 59 kD
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