Increased serum levels of granulocyte colony-stimulating factor in patients with severe congenital neutropenia.
Increased serum levels of granulocyte colony-stimulating factor in patients with severe congenital neutropenia.
复制标题
严重先天性中性粒细胞减少症患者的粒细胞集落刺激因子血清水平升高。
作者：
K. Mempel;T. Pietsch;T. Menzel;C. Zeidler;K. Welte
严重先天性中性粒细胞减少症（SCN），也称为Kostmann综合征，其特征是早幼粒细胞水平的骨髓生成成熟停止，骨髓（BM）和血液中缺乏中性粒细胞。SCN的病理生理假说包括：(1)粒细胞集落刺激因子（G-CSF）产生缺陷，和/或(2)对G-CSF的反应缺陷。为了排除G-CSF产生缺陷，我们使用Western blot分析和NFS-60增殖试验检测了SCN患者血清中G-CSF的存在。通过这些检测，我们能够检测到SCN患者血清G-CSF水平（150至910 pg/mL）比正常对照组（在未检测到和100 pg/mL之间）升高。这些结果表明，SCN患者在G-CSF生成方面没有缺陷，但中性粒细胞前体对内源性G-CSF的反应存在缺陷。
Severe congenital neutropenia (SCN), also known as Kostmann Syndrome, is characterized by a maturation arrest of myelopoiesis at the level of promyelocytes with absence of neutrophils in bone marrow (BM) and blood. Hypotheses of the pathophysiology of SCN include (1) defective production of granulocyte colony-stimulating factor (G-CSF), and/or (2) defective response to G-CSF. To exclude defective G-CSF production we tested sera from patients with SCN for the presence of G-CSF using Western blot analysis and NFS-60 proliferation assay. Using these assays we were able to detect increased G-CSF serum levels in SCN patients (150 to 910 pg/mL) as compared with normal controls (between undetectable and 100 pg/mL). These results suggest that patients with SCN have no defect in G-CSF production but a defective response of neutrophil precursors to endogenous G-CSF.
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影响因子：
158.5
作者：
BONILLA, MA;GILLIO, AP;OREILLY, RJ
通讯作者：
OREILLY, RJ
影响因子：
56.9
作者：
SOUZA, LM;BOONE, TC;WELTE, K
通讯作者：
WELTE, K
影响因子：
158.5
作者：
GABRILOVE, JL;JAKUBOWSKI, A;SOUZA, L
通讯作者：
SOUZA, L
DOI：
10.1073/pnas.82.5.1526
发表时间：
1985-01-01
影响因子：
11.1
作者：
WELTE, K;PLATZER, E;MOORE, MAS
通讯作者：
MOORE, MAS