Analysis of MCFD2- and LMAN1-deficient mice demonstrates distinct functions in vivo-Reference-Cited by-同舟云学术

Analysis of MCFD2- and LMAN1-deficient mice demonstrates distinct functions in vivo

Published:2018-05-07 Issue:9 Volume:2 Page:1014-1021
ISSN:2473-9529
Container-title:Blood Advances
language:en
Short-container-title:

Author:

Zhu Min¹²,Zheng Chunlei¹,Wei Wei¹,Everett Lesley³,Ginsburg David³⁴⁵⁶⁷,Zhang Bin¹^ORCID

Affiliation:

1. Genomic Medicine Institute, Lerner Research Institute of Cleveland Clinic, Cleveland, OH;

2. Department of Pathology, Karamay Central Hospital, Karamay, China; and

3. Life Sciences Institute,

4. Department of Internal Medicine,

5. Department of Human Genetics,

6. Department of Pediatrics and Communicable Diseases, and

7. Howard Hughes Medical Institute, University of Michigan, Ann Arbor, MI

Abstract

Key Points LMAN1 and MCFD2 have distinct functions and are required for ER-to-Golgi transport of FV, FVIII, and α1-antitrypsin. An alternative transport pathway is responsible for remaining FV/FVIII secretion in LMAN1/MCFD2-deficient mice.

Publisher

American Society of Hematology

Subject

Hematology

Link

http://ashpublications.org/bloodadvances/article-pdf/2/9/1014/880853/advances018317.pdf

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5. Bleeding due to disruption of a cargo-specific ER-to-Golgi transport complex;Zhang;Nat Genet,2003

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3. Structure of full-length ERGIC-53 in complex with MCFD2 for cargo transport;2023-08-27

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