Cited by 300Cellular transport and secretionProtein Kinase Regulation and GTPase SignalingRetinal Development and Disorders

Purification of component A of Rab geranylgeranyl transferase: possible identity with the choroideremia gene product

Seabra, Miguel C., Brown, Michael S., Slaughter, Clive A., Südhof, Thomas C., Goldstein, Joseph L.·1992·Cell

Abstract

Rab geranylgeranyl transferase (GG transferase) from rat brain contains two components, A and B. Component B comprises polypeptides of 60 and 38 kd. Here we report the purification of component A, a single 95 kd polypeptide. The holoenzyme attaches 3H-geranylgeranyl to cysteines in two GTP-binding proteins, Rab3A and Rab1A. The reaction is abolished when both cysteines in the COOH-terminal CysCys sequence of Rab1A are mutated to serines. The mutant protein inhibits transfer of 3H-geranylgeranyl to wild-type Rab1A and Rab3A, suggesting that the enzyme recognizes conserved sequences distinct from the COOH-terminus. Six peptides from rat component A show striking similarity to the product of the defective gene in choroideremia, an X-linked retinal degeneration disease. The choroideremia protein resembles Rab3A GDI, which binds Rab3A. We hypothesize that component A binds conserved sequences in Rab and that component B transfers geranylgeranyl. A defect in this reaction may cause choroideremia.

Integrity signals

Registry

Crossref · 10.1016/0092-8674(92)90253-9

Cites

31 of 31 references verified0 citation errors · 0 not checkable

Statistics

Recomputing p-values needs the paper’s full text.

Claims the paper makes

This paper’s full text isn’t openly available, so its claims and self-check weren’t run. The registry and citation checks still apply.

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

  • Choroideremia is caused by mutations in the REP1 (aka CHM) gene, which encodes RAB escort protein-1.

    “Choroideremia is caused by mutations in the REP1 (aka CHM) gene, which encodes RAB escort protein-1 (Cremers et al., 1990; Seabra et al., 1992).”

    From The cell biology of the retinal pigment epithelium (2020) · cites this paper as Seabra et al., 1992

Cites

Flagged first, then by citations

Cited by

Sorted by citations

Authors
Seabra, M. C., Brown, M. S., Slaughter, C. A., Südhof, T. C., & Goldstein, J. L.
Affiliations
  • The University of Texas Southwestern Medical Center (US)
  • Howard Hughes Medical Institute (US)
Venue
Cell
Publisher
Elsevier BV
Published
1992-09
Volume
70
Issue
6
Pages
1049-1057
ISSN
0092-8674 (ISSN-L)
OpenAlex
W2023062131
Cited by
300
References
31
Citations by year
2026: 4 · 2025: 3 · 2023: 1 · 2022: 8 · 2021: 6 · 2020: 6 · 2019: 7 · 2018: 9 · 2017: 9 · 2016: 3 · 2015: 6 · 2014: 6 · 2013: 6 · 2012: 7
FWCI
11.02
Type
journal article
Language
en
Open access
closed
Locations
  • Cell (publishedVersion)
  • PubMed (publishedVersion)
Funders
  • National Institutes of Health
  • U.S. Geological Survey
  • National Heart, Lung, and Blood Institute (HL-20948, P01HL020948)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Cellular transport and secretion
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Protein Kinase Regulation and GTPase Signaling
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Retinal Development and Disorders
Keywords
Rab geranylgeranyltransferase, choroideremia, Rab3A, Rab1A, geranylgeranylation, G proteins, cysteine mutants, X-linked retinal degeneration, protein purification
MeSH terms
Amino Acid Sequence, Animals, Base Sequence, Brain, Chromatography, Liquid, Molecular Sequence Data, Mutation, Rats, Inbred Strains, Sequence Homology, Nucleic Acid, Transferases, Choroideremia, Alkyl and Aryl Transferases, Rats

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review