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Analysis of genomic imprinting at 1p35-36 in neuroblastoma.
Med Pediatr Oncol. 2001 Jan; 36(1):52-5.MP

Abstract

BACKGROUND

Deletion of the distal short arm of chromosome 1 occurs in 25-35% of primary neuroblastomas, and a putative tumor suppressor gene has been mapped to a consensus region of deletion at 1p36.2-36.3. Indirect evidence suggests the presence of an imprinted neuroblastoma suppressor gene within this region, as well as an additional nonimprinted, proximal suppressor gene, inactivation of which correlates with MYCN amplification.

PROCEDURE

To test this hypothesis, we performed 1p loss of heterozygosity (LOH) studies on a series of neuroblastomas for which parental DNA had been collected. PCR-formatted polymorphic markers were used to determine the size of the 1p deletion and the parental origin of the deleted 1p homologue.

RESULTS

Twenty-six neuroblastomas with 1p LOH were evaluated. Twenty-four had MYCN amplification, and of these, 15 demonstrated loss of the paternally inherited 1p. Two neuroblastomas with a single copy of MYCN were evaluated and both had deletion of the paternally inherited 1p, with one case exhibiting a small terminal deletion. In addition, we have reviewed 49 previously reported neuroblastomas where 1p LOH data and the parental origin of the deleted lp homologue were available.

CONCLUSIONS

Analyzed together, these 75 neuroblastomas demonstrate random deletion of parental 1p homologues (P = 0.30). Further, tumors with smaller deletions (breakpoints distal to D1S201 or D1S7) showed a random loss of the parental 1p homologues (P = 0.59), contrary to the expected preferential maternal 1p deletion if an imprinted suppressor gene mapped to this region. However, 19 tumors with 1p LOH and single copy MYCN had deletion of the maternal 1p homologue preferentially (P = 0.02), which does not exclude the possibility that loss of an imprinted suppressor gene plays a role in this subset.

Authors+Show Affiliations

Division of Oncology, Children's Hospital of Philadelphia, Pennsylvania 19104-4318, USA. hogarty@kermit.oncol.chop.eduNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, P.H.S.
Review

Language

eng

PubMed ID

11464906

Citation

Hogarty, M D., et al. "Analysis of Genomic Imprinting at 1p35-36 in Neuroblastoma." Medical and Pediatric Oncology, vol. 36, no. 1, 2001, pp. 52-5.
Hogarty MD, Maris JM, White PS, et al. Analysis of genomic imprinting at 1p35-36 in neuroblastoma. Med Pediatr Oncol. 2001;36(1):52-5.
Hogarty, M. D., Maris, J. M., White, P. S., Guo, C., & Brodeur, G. M. (2001). Analysis of genomic imprinting at 1p35-36 in neuroblastoma. Medical and Pediatric Oncology, 36(1), 52-5.
Hogarty MD, et al. Analysis of Genomic Imprinting at 1p35-36 in Neuroblastoma. Med Pediatr Oncol. 2001;36(1):52-5. PubMed PMID: 11464906.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Analysis of genomic imprinting at 1p35-36 in neuroblastoma. AU - Hogarty,M D, AU - Maris,J M, AU - White,P S, AU - Guo,C, AU - Brodeur,G M, PY - 2001/7/24/pubmed PY - 2001/8/17/medline PY - 2001/7/24/entrez SP - 52 EP - 5 JF - Medical and pediatric oncology JO - Med Pediatr Oncol VL - 36 IS - 1 N2 - BACKGROUND: Deletion of the distal short arm of chromosome 1 occurs in 25-35% of primary neuroblastomas, and a putative tumor suppressor gene has been mapped to a consensus region of deletion at 1p36.2-36.3. Indirect evidence suggests the presence of an imprinted neuroblastoma suppressor gene within this region, as well as an additional nonimprinted, proximal suppressor gene, inactivation of which correlates with MYCN amplification. PROCEDURE: To test this hypothesis, we performed 1p loss of heterozygosity (LOH) studies on a series of neuroblastomas for which parental DNA had been collected. PCR-formatted polymorphic markers were used to determine the size of the 1p deletion and the parental origin of the deleted 1p homologue. RESULTS: Twenty-six neuroblastomas with 1p LOH were evaluated. Twenty-four had MYCN amplification, and of these, 15 demonstrated loss of the paternally inherited 1p. Two neuroblastomas with a single copy of MYCN were evaluated and both had deletion of the paternally inherited 1p, with one case exhibiting a small terminal deletion. In addition, we have reviewed 49 previously reported neuroblastomas where 1p LOH data and the parental origin of the deleted lp homologue were available. CONCLUSIONS: Analyzed together, these 75 neuroblastomas demonstrate random deletion of parental 1p homologues (P = 0.30). Further, tumors with smaller deletions (breakpoints distal to D1S201 or D1S7) showed a random loss of the parental 1p homologues (P = 0.59), contrary to the expected preferential maternal 1p deletion if an imprinted suppressor gene mapped to this region. However, 19 tumors with 1p LOH and single copy MYCN had deletion of the maternal 1p homologue preferentially (P = 0.02), which does not exclude the possibility that loss of an imprinted suppressor gene plays a role in this subset. SN - 0098-1532 UR - https://www.unboundmedicine.com/medline/citation/11464906/Analysis_of_genomic_imprinting_at_1p35_36_in_neuroblastoma_ L2 - https://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0098-1532&date=2001&volume=36&issue=1&spage=52 DB - PRIME DP - Unbound Medicine ER -