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Published in 2022 at "Biochemical Journal"
DOI: 10.1042/bcj20220055
Abstract: α1-antitrypsin (AAT) is a serine protease inhibitor synthesized in hepatocytes and protects the lung from damage by neutrophil elastase. AAT gene mutations result in AAT deficiency (AATD), which leads to lung and liver diseases. The…
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Keywords:
lman1 mcfd2;
mcfd2;
secretion;
aat ... See more keywords
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Published in 2018 at "Blood advances"
DOI: 10.1182/bloodadvances.2017014274
Abstract: Patients with RUNX1 haplodeficiency have thrombocytopenia, platelet dysfunction, and deficiencies of α-granules and dense granules. Platelet expression profiling of a patient with a heterozygous RUNX1 mutation (c.969-323G>T) revealed decreased RAB1B, which encodes a small G…
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Keywords:
rab1b;
runx1;
golgi transport;
runx1 haplodeficiency ... See more keywords
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1
Published in 2022 at "Journal of cell science"
DOI: 10.1242/jcs.260196
Abstract: Polarized transport is essential for constructing multiple plasma membrane domains in the cell. Drosophila photoreceptors are an excellent model system to study the mechanisms of polarized transport. Rab11 is the key factor regulating the post-Golgi…
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Keywords:
drosophila photoreceptors;
transport;
post golgi;
rh1 ... See more keywords
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1
Published in 2018 at "Frontiers in Cell and Developmental Biology"
DOI: 10.3389/fcell.2017.00122
Abstract: A large number of proteins are synthesized de novo in the endoplasmic reticulum (ER). They are transported through the Golgi apparatus and then delivered to their proper destinations. The ER and the Golgi play a…
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Keywords:
budding yeast;
regulation golgi;
transport;
transport dynamics ... See more keywords
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Published in 2022 at "International Journal of Molecular Sciences"
DOI: 10.3390/ijms23073590
Abstract: The Golgi complex is the central station of the secretory pathway. Knowledge about the mechanisms of intra-Golgi transport is inconsistent. Here, we compared the explanatory power of the cisterna maturation-progression model and the kiss-and-run model.…
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Keywords:
run model;
cisternal pores;
intra golgi;
model ... See more keywords