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血清小清蛋白,小鼠营养不良和肌强直中肌肉疾病的一个指标。

Serum parvalbumin, an indicator of muscle disease in murine dystrophy and myotonia.

作者信息

Jockusch H, Friedrich G, Zippel M

机构信息

Developmental Biology Unit, University of Bielefeld, Federal Republic of Germany.

出版信息

Muscle Nerve. 1990 Jun;13(6):551-5. doi: 10.1002/mus.880130613.

Abstract

The soluble Ca(++)-binding protein parvalbumin (PV) is highly concentrated in fast muscle fibers of the wild type mouse. Employing Sandwich ELISA, we have shown that PV is present in the serum of normal mice and that its level is indicative of the disease status of muscle. Elevated PV levels were found in mice with X-linked dystrophy (mdx) and reduced levels in myotonic (ADR) mice. Serum creatine kinase (CK) levels were elevated in mdx and normal in ADR mice. Because myotonic mouse muscle has a strongly reduced PV content, the reduced PV serum level in ADR mice indicated that serum PV is derived from skeletal muscle. Serum PV in mdx mice, in which muscle PV content is close to normal, is a measure of the necrosis of fast muscle fibers. Serum levels of PV and CK were not significantly elevated in heterozygous (mdx/+) carrier females. Serum PV in Duchenne patients was below the limit of detection.

摘要

可溶性钙结合蛋白小白蛋白(PV)在野生型小鼠的快肌纤维中高度富集。采用夹心酶联免疫吸附测定法,我们发现正常小鼠血清中存在PV,且其水平可指示肌肉的疾病状态。在患有X连锁肌营养不良(mdx)的小鼠中发现PV水平升高,而在强直性肌营养不良(ADR)小鼠中PV水平降低。mdx小鼠血清肌酸激酶(CK)水平升高,ADR小鼠血清CK水平正常。由于强直性肌营养不良小鼠肌肉中的PV含量大幅降低,ADR小鼠血清中PV水平降低表明血清PV来源于骨骼肌。在mdx小鼠中,其肌肉PV含量接近正常,血清PV是快肌纤维坏死的一个指标。杂合子(mdx/+)携带者雌性小鼠的血清PV和CK水平没有显著升高。杜兴氏病患者的血清PV低于检测限。

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