跳转至内容

Dear Customer:

The current international situation is complex and volatile, and uncertain tariff policies may potentially impact our product prices. Given these uncertainties, we value your understanding regarding order-related matters.

If you decide to place an order during this period, we reserve the right to adjust the price based on the evolving situation. We understand that market changes may cause inconvenience. We will negotiate with you if there’s a significant price fluctuation due to tariff policy changes before the order’s actual delivery, and in such cases we may adjust or cancel the order as necessary.

We are planning system maintenance between Friday, Apr 11 at 9:00 PM CDT and Saturday, Apr 12 at 9:00 AM CDT. This will impact both web and offline transactions, including online orders, quotes, price and availability checks, and order status inquiries. We apologize for any inconvenience.

Merck
CN
  • The OSMR Gene Is Involved in Hirschsprung Associated Enterocolitis Susceptibility through an Altered Downstream Signaling.

The OSMR Gene Is Involved in Hirschsprung Associated Enterocolitis Susceptibility through an Altered Downstream Signaling.

International journal of molecular sciences (2021-05-01)
Tiziana Bachetti, Francesca Rosamilia, Martina Bartolucci, Giuseppe Santamaria, Manuela Mosconi, Serenella Sartori, Maria Rosaria De Filippo, Marco Di Duca, Valentina Obino, Stefano Avanzini, Domenico Mavilio, Simona Candiani, Andrea Petretto, Alessio Pini Prato, Isabella Ceccherini, Francesca Lantieri
摘要

Hirschsprung (HSCR) Associated Enterocolitis (HAEC) is a common life-threatening complication in HSCR. HAEC is suggested to be due to a loss of gut homeostasis caused by impairment of immune system, barrier defense, and microbiome, likely related to genetic causes. No gene has been claimed to contribute to HAEC occurrence, yet. Genetic investigation of HAEC by Whole-Exome Sequencing (WES) on 24 HSCR patients affected (HAEC) or not affected (HSCR-only) by enterocolitis and replication of results on a larger panel of patients allowed the identification of the HAEC susceptibility variant p.H187Q in the Oncostatin-M receptor (OSMR) gene (14.6% in HAEC and 5.1% in HSCR-only, p = 0.0024). Proteomic analysis on the lymphoblastoid cell lines from one HAEC patient homozygote for this variant and one HAEC patient not carrying the variant revealed two well distinct clusters of proteins significantly up or downregulated upon OSM stimulation. A marked enrichment in immune response pathways (q < 0.0001) was shown in the HAEC H187 cell line, while proteins upregulated in the HAEC Q187 lymphoblasts sustained pathways likely involved in pathogen infection and inflammation. In conclusion, OSMR p.H187Q is an HAEC susceptibility variant and perturbates the downstream signaling cascade necessary for the gut immune response and homeostasis maintenance.

材料
货号
品牌
产品描述

Sigma-Aldrich
抗 α-微管蛋白单克隆抗体 小鼠抗, ascites fluid, clone B-5-1-2
登录查看公司和协议定价
货号包装规格是否有货价格数量
100 μL
预计发货时间 April 14, 2025
详情...
CN¥6,922.02
0.2 mL
预计发货时间 April 14, 2025
详情...
CN¥8,960.87
0.5 mL
预计发货时间 April 14, 2025
详情...
CN¥17,920.36
Sigma-Aldrich
抗 MAP 激酶,活化(二磷酸化 ERK-1&2)抗体,小鼠单克隆, clone MAPK-YT, purified from hybridoma cell culture
登录查看公司和协议定价
货号包装规格是否有货价格数量
25 μL
预计发货时间 April 14, 2025
详情...
新价格,新优惠!
CN¥1,664.93
100 μL
请联系客服了解存货情况
CN¥4,842.47
200 μL
请联系客服了解存货情况
新价格,新优惠!
CN¥6,766.60
Sigma-Aldrich
抗-VIM 兔抗, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
登录查看公司和协议定价
货号包装规格是否有货价格数量
100 μL
请联系客服了解存货情况
CN¥4,919.26
Sigma-Aldrich
Anti-RAB11FIP5 antibody produced in rabbit, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
登录查看公司和协议定价
货号包装规格是否有货价格数量
100 μL
请联系客服了解存货情况
CN¥5,551.31