RGD Reference Report - Different expression of catecholamine transporters in phaeochromocytomas from patients with von Hippel-Lindau syndrome and multiple endocrine neoplasia type 2. - Rat Genome Database

RGD Reference Report - Different expression of catecholamine transporters in phaeochromocytomas from patients with von Hippel-Lindau syndrome and multiple endocrine neoplasia type 2. - Rat Genome Database

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Different expression of catecholamine transporters in phaeochromocytomas from patients with von Hippel-Lindau syndrome and multiple endocrine neoplasia type 2.
Authors:	Huynh, TT Pacak, K Brouwers, FM Abu-Asab, MS Worrell, RA Walther, MM Elkahloun, AG Goldstein, DS Cleary, S Eisenhofer, G
Citation:	Huynh TT, etal., Eur J Endocrinol. 2005 Oct;153(4):551-63.
RGD ID:	5131200
Pubmed:	PMID:16189177 (View Abstract at PubMed)
PMCID:	PMC2288736 (View Article at PubMed Central)
DOI:	DOI:10.1530/eje.1.01987 (Journal Full-text)
OBJECTIVE: Phaeochromocytomas in patients with multiple endocrine neoplasia type 2 (MEN 2) produce adrenaline, whereas those with von Hippel-Lindau (VHL) syndrome do not. This study assessed whether these distinctions relate to differences in expression of the transporters responsible for uptake and storage of catecholamines - the noradrenaline transporter and the vesicular monoamine transporters (VMAT 1 and VMAT 2). METHODS: Tumour tissue and plasma samples were obtained from 31 patients with hereditary phaeochromocytoma - 18 with VHL syndrome and 13 with MEN 2. We used quantitative PCR, Western blotting, electron microscopy, immunohistochemistry and measurements of plasma and tumour catecholamines to assess differences in expression of the transporters in noradrenaline-producing vs adrenaline-producing hereditary tumours. These differences were compared with those in a further group of 26 patients with non-syndromic phaeochromocytoma. RESULTS: Adrenaline-producing phaeochromocytomas in MEN 2 patients expressed more noradrenaline transporter mRNA and protein than noradrenaline-producing tumours in VHL patients. In contrast, there was greater expression of VMAT 1 in VHL than MEN 2 tumours, while expression of VMAT 2 did not differ significantly. These differences were associated with larger numbers of storage vesicles and higher tissue contents of catecholamines in MEN 2 than in VHL tumours. Differences in expression of the noradrenaline transporter were weaker, and those of VMAT 1 and VMAT 2 stronger, in noradrenaline and adrenaline-producing non-syndromic than in hereditary tumours. CONCLUSIONS: The findings show that, in addition to differences in catecholamine biosynthesis, phaeochromocytomas in MEN 2 and VHL syndrome also differ in expression of the transporters responsible for uptake and vesicular storage of catecholamines.

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Object Symbol	Species	Term	Qualifier	Evidence	With	Notes	Source	Original Reference(s)
SLC18A1	Human	von Hippel-Lindau disease		IEP		mRNA:increased expression:tumor (human)	RGD
Slc18a1	Rat	von Hippel-Lindau disease		ISO	SLC18A1 (Homo sapiens)	mRNA:increased expression:tumor (human)	RGD
Slc18a1	Mouse	von Hippel-Lindau disease		ISO	SLC18A1 (Homo sapiens)	mRNA:increased expression:tumor (human)	RGD

Genes (Rattus norvegicus)


Slc18a1 (solute carrier family 18 member A1)

Genes (Mus musculus)


Slc18a1 (solute carrier family 18 (vesicular monoamine), member 1)

Genes (Homo sapiens)


SLC18A1 (solute carrier family 18 member A1)