Dataset Information

Human airway - Normal and CCDC39

ABSTRACT: Isolated cilia from air-liquid interface lung cells.

INSTRUMENT(S):

ORGANISM(S): Homo Sapiens (human)

TISSUE(S): Lung, Epithelial Cell

SUBMITTER: Susan Dutcher

LAB HEAD: Susan K. Dutcher

PROVIDER: PXD054965 | Pride | 2025-08-04

REPOSITORIES: Pride

ACCESS DATA

Dataset's files

Source:

			Action	DRS
	20190809_Brody_mutant2_dig_QC.raw	Raw
	20190809_Brody_mutant3_dig_QC.raw	Raw
	20200131_Brody03_cilla_TMT4_sample01_digest_C18_2.raw	Raw
	20200326_Brody03_cilla_TMT4_sample01_digest_C18_3.raw	Raw
	MS-CCDC39.xml	Xml

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Publications

Undocking of an extensive ciliary network induces proteostasis and cell fate switching resulting in severe primary ciliary dyskinesia.

Brody Steven L SL Pan Jiehong J Huang Tao T Xu Jian J Xu Huihui H Koenitizer Jeffrey R JR Brennan Steven K SK Nanjundappa Rashmi R Saba Thomas G TG Rumman Nisreen N Berical Andrew A Hawkins Finn J FJ Wang Xiangli X Zhang Rui R Mahjoub Moe R MR Horani Amjad A Dutcher Susan K SK

Science translational medicine 20250129 783

Primary ciliary dyskinesia is a rare monogenic syndrome that is associated with chronic respiratory disease, infertility, and laterality defects. Although more than 50 genes causative of primary ciliary dyskinesia have been identified, variants in the genes encoding coiled-coil domain-containing 39 (CCDC39) and CCDC40 in particular cause severe disease that is not explained by loss of ciliary motility alone. Here, we sought to understand the consequences of these variants on cellular functions b ...[more]

PMID: 39879322

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