Expression of the neural intermediate filament proteins peripherin and neurofilament-66/α-internexin in neuroblastoma

John Foley, D. Witte, F. C. Chiu, L. M. Parysek

Research output: Contribution to journalArticle

24 Citations (Scopus)

Abstract

BACKGROUND: Peripherin and neurofilament (NF)-66/α-internexin are recently characterized, neuron-specific intermediate filament proteins that are expressed in the developing peripheral nervous system. Peripherin, in particular, is highly enriched in neuronal derivatives of the neural crest. We speculated that these intermediate filament proteins would be expressed in neuroblastoma (NB), a neural crest-derived tumor with many neuronal features. EXPERIMENTAL DESIGN: By use of antibodies specific to peripherin and NF- 66/α-internexin, we detected these proteins on Western blots of NB tissue extracts and in paraffin sections of NBs. RESULTS: Western blotting indicated that NB tumor extracts contained immunoreactive proteins that co-migrated with rat peripherin and human NF-66/α-internexin from normal tissues, thus establishing the specificity of the antibodies for these proteins in tumors. The antibody specific for peripherin labeled all NBs, including immature NBs, composite ganglioneuroblastomas and ganglioneuromas. In contrast, the NF- 66/α-internexin antibody labeled only 50% of NBs, and only weakly labeled most ganglioneuroblastomas and ganglioneuromas. Neither antibody labeled other small blue cell tumors such as lymphomas, rhabdomyosarcomas, Wilms' tumors, and Ewing sarcomas. CONCLUSIONS: The specificity of the peripherin labeling of NB and the ability of the peripherin antibody to label the entire spectrum of NBs, including ganglioneuroblastomas and ganglioneuromas, indicate that this intermediate filament protein has potential as a diagnostic marker for these related neural crest neoplasms.

Original languageEnglish (US)
Pages (from-to)193-199
Number of pages7
JournalLaboratory Investigation
Volume71
Issue number2
StatePublished - 1994
Externally publishedYes

Fingerprint

Peripherins
Intermediate Filament Proteins
Neuroblastoma
Ganglioneuroblastoma
Ganglioneuroma
Antibodies
Neural Crest
Neoplasms
Western Blotting
Proteins
Ewing's Sarcoma
Tissue Extracts
Antibody Specificity
Wilms Tumor
Rhabdomyosarcoma
Peripheral Nervous System
alpha-internexin
Paraffin
Lymphoma
Neurons

Keywords

  • Ganglioneuroblastoma
  • Ganglioneuroma

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Expression of the neural intermediate filament proteins peripherin and neurofilament-66/α-internexin in neuroblastoma. / Foley, John; Witte, D.; Chiu, F. C.; Parysek, L. M.

In: Laboratory Investigation, Vol. 71, No. 2, 1994, p. 193-199.

Research output: Contribution to journalArticle

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N2 - BACKGROUND: Peripherin and neurofilament (NF)-66/α-internexin are recently characterized, neuron-specific intermediate filament proteins that are expressed in the developing peripheral nervous system. Peripherin, in particular, is highly enriched in neuronal derivatives of the neural crest. We speculated that these intermediate filament proteins would be expressed in neuroblastoma (NB), a neural crest-derived tumor with many neuronal features. EXPERIMENTAL DESIGN: By use of antibodies specific to peripherin and NF- 66/α-internexin, we detected these proteins on Western blots of NB tissue extracts and in paraffin sections of NBs. RESULTS: Western blotting indicated that NB tumor extracts contained immunoreactive proteins that co-migrated with rat peripherin and human NF-66/α-internexin from normal tissues, thus establishing the specificity of the antibodies for these proteins in tumors. The antibody specific for peripherin labeled all NBs, including immature NBs, composite ganglioneuroblastomas and ganglioneuromas. In contrast, the NF- 66/α-internexin antibody labeled only 50% of NBs, and only weakly labeled most ganglioneuroblastomas and ganglioneuromas. Neither antibody labeled other small blue cell tumors such as lymphomas, rhabdomyosarcomas, Wilms' tumors, and Ewing sarcomas. CONCLUSIONS: The specificity of the peripherin labeling of NB and the ability of the peripherin antibody to label the entire spectrum of NBs, including ganglioneuroblastomas and ganglioneuromas, indicate that this intermediate filament protein has potential as a diagnostic marker for these related neural crest neoplasms.

AB - BACKGROUND: Peripherin and neurofilament (NF)-66/α-internexin are recently characterized, neuron-specific intermediate filament proteins that are expressed in the developing peripheral nervous system. Peripherin, in particular, is highly enriched in neuronal derivatives of the neural crest. We speculated that these intermediate filament proteins would be expressed in neuroblastoma (NB), a neural crest-derived tumor with many neuronal features. EXPERIMENTAL DESIGN: By use of antibodies specific to peripherin and NF- 66/α-internexin, we detected these proteins on Western blots of NB tissue extracts and in paraffin sections of NBs. RESULTS: Western blotting indicated that NB tumor extracts contained immunoreactive proteins that co-migrated with rat peripherin and human NF-66/α-internexin from normal tissues, thus establishing the specificity of the antibodies for these proteins in tumors. The antibody specific for peripherin labeled all NBs, including immature NBs, composite ganglioneuroblastomas and ganglioneuromas. In contrast, the NF- 66/α-internexin antibody labeled only 50% of NBs, and only weakly labeled most ganglioneuroblastomas and ganglioneuromas. Neither antibody labeled other small blue cell tumors such as lymphomas, rhabdomyosarcomas, Wilms' tumors, and Ewing sarcomas. CONCLUSIONS: The specificity of the peripherin labeling of NB and the ability of the peripherin antibody to label the entire spectrum of NBs, including ganglioneuroblastomas and ganglioneuromas, indicate that this intermediate filament protein has potential as a diagnostic marker for these related neural crest neoplasms.

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