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Selected Publication:
Pfragner, R; Behmel, A; Smith, DP; Ponder, BA; Wirnsberger, G; Rinner, I; Porta, S; Henn, T; Niederle, B.
First continuous human pheochromocytoma cell line: KNA. Biological, cytogenetic and molecular characterization of KNA cells.
J Neurocytol. 1998; 27(3):175-186
Doi: 10.1023%2FA%3A1006959625068
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- Leading authors Med Uni Graz
- Pfragner Roswitha
- Co-authors Med Uni Graz
- Porta Sepp
- Wirnsberger Gerhard
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- Abstract:
- Pheochromocytomas are rare tumours, with an incidence of 1-2 per million which arise from chromaffin cells of the adrenal medulla. They occur sporadically or as part of dominantly inherited cancer syndromes like multiple endocrine neoplasia 2 (MEN2A and 2B) and others. Continuous cell lines, not available so far, are essential tools for studies in these tumours. A continuous cell line (KNA) was established from a sporadic pheochromocytoma of the right adrenal gland of a 73-year-old woman. The KNA cells grow as suspensions of spheroids and show the morphological and immunocytochemical characteristics of neuronal chromaffin cells, such as neuroendocrine granules, and positive reactions to chromogranin- and related peptide-, neuron specific enolase and vasoactive intestinal peptide antibodies. Neurite-like processes are formed after addition of nerve growth factor. Chromosomal analyses revealed a diploid (46,XX,n = 50) to hypodiploid (43-45,XX,n = 15) karyotype. In hypodiploid metaphases most frequently #19, #17, #21 and #22 were missing. Chromosome arms 1p and 4q showed apparently consistent interstitial deletions: 6q, 8q, 13q and 22q showed clonal interstitial deletions. The cell line shows a heterozygous sequence variant TGC (cysteine) to TGG (tryptophan) in codon 611 in exon 10 of the RET proto-oncogene. So far, PC-12, a rat adrenal pheochromocytoma, has been the only continuous pheochromocytoma cell line available. KNA represents the first report on a human continuous pheochromocytoma cell line, the first report of structural chromosome aberrations in pheochromocytomas and the first report of a RET mutation TGC to TGG in exon 10 of the RET proto-oncogene in a sporadic pheochromocytoma.
- Find related publications in this database (using NLM MeSH Indexing)
- Adrenal Gland Neoplasms - genetics
- Aged - genetics
- Amino Acid Substitution - genetics
- Animals - genetics
- Catecholamines - analysis
- Chromogranin A - analysis
- Chromogranins - analysis
- Chromosome Aberrations - analysis
- Cytoplasmic Granules - chemistry
- Dopamine beta-Hydroxylase - analysis
- Drosophila Proteins - analysis
- Exons - genetics
- Female - genetics
- Humans - genetics
- Male - genetics
- Mice - genetics
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- Neoplasm Proteins - genetics
- Neoplasm Transplantation - genetics
- Nerve Growth Factor - pharmacology
- Neuropeptides - analysis
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- Ploidies - genetics
- Point Mutation - genetics
- Proto-Oncogene Proteins - genetics
- Proto-Oncogene Proteins c-ret - genetics
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- Receptor Protein-Tyrosine Kinases - genetics
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- Tumor Markers, Biological - analysis