Reactome | Removal of aminoterminal propeptides from gamma-carboxylated proteins

Removal of aminoterminal propeptides from gamma-carboxylated proteins

Stable Identifier

R-HSA-159782

Type

Pathway

Species

Homo sapiens

Compartment

Golgi lumen

ReviewStatus

5/5

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Removal of aminoterminal propeptides from gamma-carboxylated proteins

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Furin is an endopeptidase localized to the Golgi membrane that cleaves many proteins on the carboxyterminal side of the sequence motif Arg-[any residue]-(Lys or Arg)-Arg (Jones et al. 1995; Leduc et al. 1992). In the case of gamma-carboxylated proteins, if this cleavage does not occur, the proteins are still secreted but do not function properly (Bristol et al. 1993; Lind et al. 1997; Wasley et al. 1993). The aminoterminal fragments, "propeptides", generated in this reaction have no known function; the carboxylated, cleaved proteins are delivered to the cell membrane or secreted from the cell.

Literature References

PubMed ID	Title	Journal	Year
8463288	Propeptide processing during factor IX biosynthesis. Effect of point mutations adjacent to the propeptide cleavage site. Bristol, JA, Furie, BC, Furie, B	J Biol Chem	1993
1629222	Activation of human furin precursor processing endoprotease occurs by an intramolecular autoproteolytic cleavage Leduc, R, Molloy, S S, Thorne, BA, Thomas, G	J Biol Chem	1992
8473289	PACE/furin can process the vitamin K-dependent pro-factor IX precursor within the secretory pathway Wasley, LC, Rehemtulla, A, Bristol, JA, Kaufman, RJ	J. Biol. Chem.	1993
9108399	Naturally occurring Arg(-1) to His mutation in human protein C leads to aberrant propeptide processing and secretion of dysfunctional protein C Lind, B, Johnsen, AH, Thorsen, S	Blood	1997
8846780	Intracellular trafficking of furin is modulated by the phosphorylation state of a casein kinase II site in its cytoplasmic tail Jones, BG, Thomas, L, Molloy, S S, Thulin, CD, Fry, MD, Walsh, KA, Thomas, G	EMBO J	1995