
The fate of newly synthesized V-ATPase accessory subunit Ac45
in the secretory pathway
Vincent Th. G. Schoonderwoert, Eric J. R. Jansen and Gerard J. M. Martens
Department of Animal Physiology, Nijmegen Center for Molecular Life Sciences, University of Nijmegen, the Netherlands
The vacuolar H
+
-ATPase (V-ATPase) is a multimeric
enzyme complex that acidifies organelles of the vacuolar
system in eukaryotic cells. Proteins that interact with the
V-ATPase may play an important role in controlling the
intracellular localization and activity of the proton pump.
The neuroendocrine-enriched V-ATPase accessory subunit
Ac45 may represent such a protein as it has been shown to
interact with the membrane sector of the V-ATPase in only a
subset of organelles. Here, we examined the fate of newly
synthesized Ac45 in the secretory pathway of a neuroendo-
crine cell. A major portion of intact 46-kDa Ac45 was
found to be N-linked glycosylated to 62 kDa and a minor
fraction to 64 kDa. Trimming of the N-linked glycans
gave rise to glycosylated Ac45-forms of 61 and 63 kDa
that are cleaved to a C-terminal fragment of 42–44 kDa (the
deglycosylated form is 23 kDa), and a previously not
detected 22-kDa N-terminal cleavage fragment (the
deglycosylated form is 20 kDa). Degradation of the
N-terminal fragment is rapid, does not occur in lysosomes
and is inhibited by brefeldin A. Both the N- and C-terminal
fragment pass the medial Golgi, as they become partially
endoglycosidase H resistant. The Ac45 cleavage event is a
relatively slow process (half-life of intact Ac45 is 4–6 h) and
takes place in the early secretory pathway, as it is not affected
by brefeldin A and monensin. Tunicamycin inhibited
N-linked glycosylation of Ac45 and interfered with the
cleavage process, suggesting that Ac45 needs proper folding
for the cleavage to occur. Together, our results indicate that
Ac45 folding and cleavage occur slowly and early in the
secretory pathway, and that the cleavage event may be linked
to V-ATPase activation.
Keywords: acidification; regulated secretory pathway; post-
translational modification; vacuolar proton ATPase;
Xenopus.
Acidification of organelles in eukaryotic cells is required for
a variety of cellular processes, such as the release of ligands
from receptors during endocytosis and the hydrolysis of
macromolecules in lysosomes [1–3]. In the secretory path-
way, the lumen gradually acidifies from endoplasmic
reticulum (ER) to Golgi to secretory granules (reviewed in
[4]). The pH of the lumen of the ER, Golgi, and trans-Golgi
Network (TGN) is 7.3, 6.4, and 6.0, respectively, and
is similar in regulated and nonregulated secretory cells
[5–10]. The significance of the pH in the ER remains to be
established, although it seems likely that ER processes such
as protein glycosylation and folding depend on it. The low
pH in the Golgi has been shown to be important for the
regulation of protein–protein interactions [11,12] and the
activity of the N-glycan processing enzyme sialyltransferase
[13]. In the TGN, an acidic pH is necessary for the proper
processing of proproteins [14] and for the condensation of
regulated secretory proteins, which is important for their
targeting to immature secretory granules [15–17]. Immature
secretory granules mature and become progressively more
acidic (pH of 5.5 [18–20]). Granular acidification further
concentrates regulated proteins [21], while nonregulated
proteins are sorted away into clathrin-coated vesicles that
pinch off from the maturing granule [22–24]. Furthermore,
the acidic granular pH is necessary for the processing
enzymes to efficiently cleave the prohormones [25].
Acidification of intracellular compartments is established
and maintained by the vacuolar H
+
-ATPase (V-ATPase).
This multimeric enzyme complex consists of at least 13
different subunits that have been classified into a membrane
integral sector (V
0
) and a peripheral sector (V
1
) [26,27]. The
V-ATPase V
1
sector contains the catalytic site which
hydrolyses ATP to translocate protons across the mem-
brane by the proton-pore forming V
0
sector. In the ER, the
assembly of the V-ATPase starts with the V
0
-sector and may
be completed in this compartment by the build-up of the V
1
onto the V
0
[28,29]. Given the pH in the ER, the V-ATPase
should be considered as being essentially inactive in this part
of the secretory pathway. An active V-ATPase is required
further downstream in the secretory pathway but it is not
known in which compartment the V-ATPase becomes
active and which mechanism is involved in the targeting of
the V-ATPase to the various secretory pathway compart-
ments. V-ATPase interacting proteins, such as the accessory
subunit Ac45, may play an important role in this targeting
process, as Ac45 has been shown to interact with the
Correspondence to G. J. M. Martens, Department of Animal Physi-
ology, Nijmegen Center for Molecular Life Sciences, University of
Nijmegen, Geert Grooteplein Zuid 28, 193RT, 6525 GA Nijmegen,
the Netherlands. Fax: + 31 24 3615317, Tel.: + 31 24 3610564,
E-mail: g.martens@ncmls.kun.nl
Abbreviations: Baf, bafilomycin A1; BFA, brefeldin A; EndoH,
endoglycosidase H; ER, endoplasmic reticulum; NDGA, nordi-
hydroguaiaretic acid; NIL, neurointermediate lobe; PC2, prohormone
convertase 2; POMC, proopiomelanocortin; TGN, trans-Golgi net-
work; V-ATPase, vacuolar H
+
-ATPase.
Note: a web page is available at
http://www.kun.nl/molanphys/Homepage/home.htm
(Received 26 October 2001, revised 6 February 2002, accepted 8
February 2002)
Eur. J. Biochem. 269, 1844–1853 (2002) ÓFEBS 2002 doi:10.1046/j.1432-1033.2002.02831.x