MARC details
| 000 -LEADER |
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| fixed length control field |
03269nam a22004577a 4500 |
| 008 - FIXED-LENGTH DATA ELEMENTS--GENERAL INFORMATION |
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| fixed length control field |
181102s20182018 xxu||||| |||| 00| 0 eng d |
| 022 ## - INTERNATIONAL STANDARD SERIAL NUMBER |
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| International Standard Serial Number |
2046-1402 |
| 024 ## - OTHER STANDARD IDENTIFIER |
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| Standard number or code |
10.12688/f1000research.14568.1 [doi] |
| 024 ## - OTHER STANDARD IDENTIFIER |
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| Standard number or code |
PMC6173107 [pmc] |
| 040 ## - CATALOGING SOURCE |
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| Original cataloging agency |
Ovid MEDLINE(R) |
| 099 ## - LOCAL FREE-TEXT CALL NUMBER (OCLC) |
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| PMID |
30345003 |
| 245 ## - TITLE STATEMENT |
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| Title |
New insights on the pathogenesis of paraganglioma and pheochromocytoma. [Review] |
| 251 ## - Source |
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| Source |
F1000Research. 7, 2018. |
| 252 ## - Abbreviated Source |
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| Abbreviated source |
F1000Res. 7, 2018. |
| 253 ## - Journal Name |
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| Journal name |
F1000Research |
| 260 ## - PUBLICATION, DISTRIBUTION, ETC. |
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| Year |
2018 |
| 260 ## - PUBLICATION, DISTRIBUTION, ETC. |
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| Manufacturer |
FY2019 |
| 265 ## - SOURCE FOR ACQUISITION/SUBSCRIPTION ADDRESS [OBSOLETE] |
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| Publication status |
epublish |
| 266 ## - Date added to catalog |
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| Date added to catalog |
2018-11-02 |
| 520 ## - SUMMARY, ETC. |
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| Abstract |
Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare chromaffin cell tumors (PPGLs) that at times raise significant challenges in clinical recognition, diagnosis, and therapy and when undiagnosed could associate with severe morbidity. Recent discoveries in PPGL genetics propelled our understanding in the pathophysiology of tumorigenesis and allowed the application of functional classification of pathogenetically distinct groups of PPGLs. This also resulted in a qualitative change in our approach to clinical assessment, diagnosis, and therapy of different subgroups of PPGLs. Establishment of the fact that mutations in multiple components of the PHD-VHL-HIF-2alpha pathway associate with pseudohypoxia-driven tumorigenesis allowed us not only to better understand the effect of this phenomenon but also to more deeply appreciate the value of functional abnormalities in the physiologic tissue oxygen-sensing mechanism. Mutations in the tricarboxylic acid cycle-related genes opened an additional window into understanding the physiology of one of the basic cellular metabolic pathways and consequences of its disruption. Mutations in the kinase signaling-related genes allow the PPGL field to join a massive innovative process in therapeutic advances in current oncology. New pathophysiologically distinct groups of mutations will widen and deepen our understanding of additional pathways in PPGL tumorigenesis and hopefully introduce additional diagnostic and therapeutic approaches. All of these developments are tremendously important in our understanding of both the normal physiology and pathophysiology of PPGLs and are strong tools and stimuli in the development of modern approaches to all components of medical management. |
| 546 ## - LANGUAGE NOTE |
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| Language note |
English |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
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| Topical term or geographic name entry element |
*Paraganglioma/pa [Pathology] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
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| Topical term or geographic name entry element |
*Pheochromocytoma/pa [Pathology] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
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| Topical term or geographic name entry element |
Citric Acid Cycle/ge [Genetics] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
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| Topical term or geographic name entry element |
Humans |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
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| Topical term or geographic name entry element |
Metabolic Networks and Pathways/ge [Genetics] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
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| Topical term or geographic name entry element |
Mutation |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
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| Topical term or geographic name entry element |
Paraganglioma/di [Diagnosis] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
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| Topical term or geographic name entry element |
Paraganglioma/ge [Genetics] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
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| Topical term or geographic name entry element |
Pheochromocytoma/di [Diagnosis] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
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| Topical term or geographic name entry element |
Pheochromocytoma/ge [Genetics] |
| 651 ## - SUBJECT ADDED ENTRY--GEOGRAPHIC NAME |
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| Institution |
MedStar Washington Hospital Center |
| 656 ## - INDEX TERM--OCCUPATION |
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| Department |
Endocrinology |
| 657 ## - INDEX TERM--FUNCTION |
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| Medline publication type |
Journal Article |
| 657 ## - INDEX TERM--FUNCTION |
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| Medline publication type |
Review |
| 700 ## - ADDED ENTRY--PERSONAL NAME |
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| Local Authors |
Kantorovich, Vitaly |
| 790 ## - Authors |
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| All authors |
Kantorovich V, Pacak K |
| 856 ## - ELECTRONIC LOCATION AND ACCESS |
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| DOI |
<a href="https://dx.doi.org/10.12688/f1000research.14568.1">https://dx.doi.org/10.12688/f1000research.14568.1</a> |
| Public note |
https://dx.doi.org/10.12688/f1000research.14568.1 |
| 858 ## - ORCID |
|---|
| ORCID text |
Kantorovich, Vitaly |
| Orcid |
<a href="https://orcid.org/0000-0003-2220-4904">https://orcid.org/0000-0003-2220-4904</a> |
| Name |
https://orcid.org/0000-0003-2220-4904 |
| 942 ## - ADDED ENTRY ELEMENTS (KOHA) |
|---|
| Koha item type |
Journal Article |
| Item type description |
Article |
