Literature DB >> 11405353

Garrod's foresight; our hindsight.

C R Scriver1.   

Abstract

Archibald Edward Garrod introduced a paradigm, new for its day, in medicine: Biochemistry is dynamic and different from the static nature of organic chemistry. It led him to think about metabolic pathways and to recognize that variation in Mendelian heredity could explain an 'inborn error of metabolism'. At the time, Garrod had no idea about the nature of a gene. Genes are now well understood, genomes are being described for one organism after another (including H. sapiens) and it is understood that genomes 'speak biochemistry (not phenotype)'. Accordingly, in the era of genomics, biochemistry and physiology become the bases of functional genomics and it is possible to appreciate why 'nothing in biology makes sense without evolution' (and nothing in medicine will make sense without biology). Mendelian, biochemical and molecular genetics together have revealed what lies behind the four canonical inborn errors described by Garrod (albinism, alkaptonuria, cystinuria and pentosuria). Both older and newer ideas in genetics, new tools for applying them, and renewed respect for the clinician-scientist will enhance our understanding of the human biological variation that accounts for variant states of health and overt disease; an 'unsimple' phenotype (phenylketonuria) is used to illustrate in some detail. What can be known and what ought to be done with knowledge about human genetics to benefit individuals, families and communities (society) is both opportunity and challenge.

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Year:  2001        PMID: 11405353     DOI: 10.1023/a:1010351630856

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN: 0141-8955            Impact factor:   4.982


  52 in total

1.  Genomics: journey to the center of biology.

Authors:  E S Lander; R A Weinberg
Journal:  Science       Date:  2000-03-10       Impact factor: 47.728

Review 2.  Antagonists to the rescue.

Authors:  W J Welch; M Howard
Journal:  J Clin Invest       Date:  2000-04       Impact factor: 14.808

3.  Molecular structure of nucleic acids; a structure for deoxyribose nucleic acid.

Authors:  J D WATSON; F H CRICK
Journal:  Nature       Date:  1953-04-25       Impact factor: 49.962

4.  Genetics: voyage of discovery for Everyman. (Presidential address to the Society for Pediatric Research, April 29, 1976, St. Louis, Missouri).

Authors:  C R Scriver
Journal:  Pediatr Res       Date:  1976-10       Impact factor: 3.756

5.  Alkaptonuria: such a long journey.

Authors:  C R Scriver
Journal:  Nat Genet       Date:  1996-09       Impact factor: 38.330

6.  Structural and functional analysis of mutations in alkaptonuria.

Authors:  J M Rodríguez; D E Timm; G P Titus; D Beltrán-Valero De Bernabé; O Criado; H A Mueller; S Rodríguez De Córdoba; M A Peñalva
Journal:  Hum Mol Genet       Date:  2000-09-22       Impact factor: 6.150

Review 7.  Life with 6000 genes.

Authors:  A Goffeau; B G Barrell; H Bussey; R W Davis; B Dujon; H Feldmann; F Galibert; J D Hoheisel; C Jacq; M Johnston; E J Louis; H W Mewes; Y Murakami; P Philippsen; H Tettelin; S G Oliver
Journal:  Science       Date:  1996-10-25       Impact factor: 47.728

8.  An ongoing debate over phenylalanine hydroxylase deficiency in phenylketonuria.

Authors:  C R Scriver
Journal:  J Clin Invest       Date:  1998-06-15       Impact factor: 14.808

9.  Non-type I cystinuria caused by mutations in SLC7A9, encoding a subunit (bo,+AT) of rBAT.

Authors:  L Feliubadaló; M Font; J Purroy; F Rousaud; X Estivill; V Nunes; E Golomb; M Centola; I Aksentijevich; Y Kreiss; B Goldman; M Pras; D L Kastner; E Pras; P Gasparini; L Bisceglia; E Beccia; M Gallucci; L de Sanctis; A Ponzone; G F Rizzoni; L Zelante; M T Bassi; A L George; M Manzoni; A De Grandi; M Riboni; J K Endsley; A Ballabio; G Borsani; N Reig; E Fernández; R Estévez; M Pineda; D Torrents; M Camps; J Lloberas; A Zorzano; M Palacín
Journal:  Nat Genet       Date:  1999-09       Impact factor: 38.330

Review 10.  Comparison of the complete protein sets of worm and yeast: orthology and divergence.

Authors:  S A Chervitz; L Aravind; G Sherlock; C A Ball; E V Koonin; S S Dwight; M A Harris; K Dolinski; S Mohr; T Smith; S Weng; J M Cherry; D Botstein
Journal:  Science       Date:  1998-12-11       Impact factor: 47.728

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  5 in total

Review 1.  Garrod's Croonian Lectures (1908) and the charter 'Inborn Errors of Metabolism': albinism, alkaptonuria, cystinuria, and pentosuria at age 100 in 2008.

Authors:  Charles R Scriver
Journal:  J Inherit Metab Dis       Date:  2008-10-12       Impact factor: 4.982

Review 2.  After the genome--the phenome?

Authors:  C R Scriver
Journal:  J Inherit Metab Dis       Date:  2004       Impact factor: 4.982

Review 3.  Does hereditary metabolic disease modulate senescence and ageing?

Authors:  C R Scriver
Journal:  J Inherit Metab Dis       Date:  2002-05       Impact factor: 4.982

Review 4.  Legacies of Garrod's brilliance. One hundred years--and counting.

Authors:  L E Rosenberg
Journal:  J Inherit Metab Dis       Date:  2008-10-05       Impact factor: 4.982

Review 5.  Chemical individuality: concept and outlook.

Authors:  W A Gahl
Journal:  J Inherit Metab Dis       Date:  2008-10-07       Impact factor: 4.982

  5 in total

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