Concepedia

Porphobilinogen Synthase Function

Porphobilinogen Synthase Function is a biochemical enzymatic function belonging to the class of metabolic catalytic activities, specifically characterized by the asymmetric condensation of two molecules of 5-aminolevulinic acid to form porphobilinogen, a key committed step in the heme biosynthesis pathway within porphyrin metabolism.

254

Publications

10K

Citations

755

Authors

252

Institutions

Publications per year

2017–2026

15

Variants

Ala Dehydratase FunctionAminolevulinate Dehydratase Function

Parents

Publications

254

Phase 3 Trial of RNAi Therapeutic Givosiran for Acute Intermittent Porphyria

Manisha Balwani, Eliane Sardh, Paolo Ventura et al. · New England Journal of Medicine · 2020 · 592 citations · Full text

, , +14

On the enzymic defects in hereditary tyrosinemia.

Bengt Lindblad, Sandra Lindstedt, Göran O. Steen · Proceedings of the National Academy of Sciences · 1977 · 456 citations · Full text

, , +14

Intermittent Acute Porphyria — Demonstration of a Genetic Defect in Porphobilinogen Metabolism

Urs Meyer, Lars Strand, M. Doss et al. · New England Journal of Medicine · 1972 · 217 citations

, , +10

Porphobilinogen deaminase deficiency in mice causes a neuropathy resembling that of human hepatic porphyria

Raija L.P. Lindberg, Catherine Porcher, Bernard Grandchamp et al. · Nature Genetics · 1996 · 160 citations

, , +9

RNAi-mediated silencing of hepatic <i>Alas1</i> effectively prevents and treats the induced acute attacks in acute intermittent porphyria mice

Makiko Yasuda, Lin Gan, Brenden Chen et al. · Proceedings of the National Academy of Sciences · 2014 · 126 citations · Full text

, , +17

1–5 of 254

Authors

755

  1. EK
    Eileen K. Jaffe

    Fox Chase Cancer Center, United States

    32 publications · 1.3K citations

    25

    h-index

  2. MD
    M. Doss

    Philipps University of Marburg, Germany

    14 publications · 525 citations

    13

    h-index

  3. AR
    Alan R. Battersby

    University of Cambridge, United Kingdom

    12 publications · 345 citations

    11

    h-index

  4. GH
    Graham Hart

    University College London, United Kingdom

    8 publications · 268 citations

    8

    h-index

  5. FJ
    Finian J. Leeper

    University of Cambridge, United Kingdom

    8 publications · 177 citations

    8

    h-index

1–5 of 755

Institutions

252

  1. Fox Chase Cancer Center

    Philadelphia, United States

    20 h-index

    97 publications

    3.9K citations

  2. Philipps University of Marburg

    Marburg, Germany

    13 h-index

    40 publications

    972 citations

  3. University of Cambridge

    Cambridge, United Kingdom

    9 h-index

    34 publications

    1.1K citations

  4. Icahn School of Medicine at Mount Sinai

    New York, United States

    9 h-index

    43 publications

    3.5K citations

  5. University of Pennsylvania

    Philadelphia, United States

    8 h-index

    13 publications

    469 citations

  6. University of Utah

    Salt Lake City, United States

    7 h-index

    8 publications

    1.7K citations

  7. University of Southampton

    Southampton, United Kingdom

    6 h-index

    12 publications

    660 citations

  8. Karolinska University Hospital

    Stockholm, Sweden

    6 h-index

    46 publications

    20.5K citations

1–8 of 252

Venues

  1. Journal of Biological Chemistry

    JBC · 24 publications · 1.2K citations

    20

    h-index

  2. Biochemistry

    12 publications · 403 citations

    12

    h-index

  3. Biochemical Journal

    BJ · 11 publications · 489 citations

    11

    h-index

  4. Proceedings of the National Academy of Sciences

    PNAS · 9 publications · 939 citations

    9

    h-index

  5. Human Genetics

    8 publications · 201 citations

    8

    h-index

1–5