A animal model for Waardenburg syndrome type 4. Waardenburg syndrome (WS) is a hereditary auditory-pigmentary syndrome with hearing impairment and pigment anomaly, and the WS type 4 is associated with Hirschsprung disease. WS4 mutant mice were spontaneously arose in a breeding colony with BALB/c and MSM mixed background. Homozygous mutant mice show piebald and deafness, and most of them die between 2 and 4 weeks after birth due to megacolon. The results of allelism tests indicated that WS4 is allelic with Ednrb mutant mice (piebald-lethal and JF1). Developed by Yoshibumi Matsushima, Saitama Cancer Center. Spontaneous mutation arose in BALB/c and MSM background. The mutant mice were backcrossed to MSM/Ms. RBRC00763 条件を付加しない。 Mouse model for Waardenburg syndrome type 4-Hirschsprung disease type 2 ワーデンブルグ症候群4型-ヒルシュッスプルング病2型モデルマウス 松島　芳文 Yoshibumi MATSUSHIMA <a href='https://brc.riken.jp/mus/pcr00763'>Genotyping protocol -PCR-</a> 埼玉県立がんセンター研究所 WS4 MSM.C-Ednrb<WS4>/StmRbrc Necessary documents for ordering:<ol><li>Order form (<A HREF="https://mus.brc.riken.jp/ja/wp-content/uploads/form/form_4.docx">Japanese</A> / <A HREF="https://mus.brc.riken.jp/en/wp-content/uploads/form/form_b.docx">English</A>)</li><li>Category I MTA: MTA for distribution with RIKEN BRC (<A HREF="https://mus.brc.riken.jp/ja/wp-content/uploads/form/form_5.docx">Japanese</A> / <A HREF="https://mus.brc.riken.jp/en/wp-content/uploads/form/form_c.docx">English</A>)</li></ol> BALB/cとMSM/Msとの交配実験中に発見されたエンドセリン受容体B遺伝子 (Ednrb) の自然突然変異。エンドセリン受容体B遺伝子に生じた変異により、大結腸症、難聴、色素異常を呈し、ホモ個体のほとんどが巨大結腸のため2-4週齢で死亡する (まれに繁殖可能月齢まで生存する)。ワーデンブルグ症候群4型-ヒルシュッスプルング病2型のモデルマウス。バックグラウンドがMSMになると生存しやすい。 埼玉がんセンター研究所 松島芳文先生。(BALB/c x MSM) F1メスとMSM (CMSMF1) オスとの交配仔に出現した突然変異。MSMコンジェニック系統。 Heterozygote x Wild-type [MSM/Ms] Heterozygote x Wild-type [MSM/Ms] Mouse model for Waardenburg syndrome type 4-Hirschsprung disease type 2, WS4 Research Institute for Clinical Oncology, Saitama Cancer Center C（3〜6か月） C (3-6 months) No specific terms and conditions. (The DEPOSITOR waives its own rights under any patents, intellectual property, or other proprietary rights with respect to the results to be obtained by use of the BIOLOGICAL RESOURCE.) ワーデンブルグ症候群4型-ヒルシュッスプルング病2型モデルマウス, WS4 false Mouse Models for Human Disease