Gideon Dreyfuss is an American

biochemist Biochemists are scientists who are trained in biochemistry. They study chemical processes and chemical transformations in living organisms. Biochemists study DNA, proteins and Cell (biology), cell parts. The word "biochemist" is a portmanteau of ...

, the Isaac Norris Professor of Biochemistry and Biophysics at the

University of Pennsylvania School of Medicine The Perelman School of Medicine, commonly known as Penn Med, is the medical school of the University of Pennsylvania, a private research university in Philadelphia, Pennsylvania. Founded in 1765, the Perelman School of Medicine is the oldest medi ...

, and an investigator of the

Howard Hughes Medical Institute The Howard Hughes Medical Institute (HHMI) is an American non-profit medical research organization based in Chevy Chase, Maryland. It was founded in 1953 by Howard Hughes, an American business magnate, investor, record-setting pilot, engineer, fil ...

. He was elected to the

National Academy of Sciences The National Academy of Sciences (NAS) is a United States nonprofit, non-governmental organization. NAS is part of the National Academies of Sciences, Engineering, and Medicine, along with the National Academy of Engineering (NAE) and the Nati ...

in 2012. Dreyfuss received his Ph.D. in biological chemistry in 1978 from

Harvard University Harvard University is a private Ivy League research university in Cambridge, Massachusetts. Founded in 1636 as Harvard College and named for its first benefactor, the Puritan clergyman John Harvard, it is the oldest institution of higher le ...

and is a fellow of the

American Academy of Arts and Sciences The American Academy of Arts and Sciences (abbreviation: AAA&S) is one of the oldest learned societies in the United States. It was founded in 1780 during the American Revolution by John Adams, John Hancock, James Bowdoin, Andrew Oliver, and ...

Research

The Dreyfuss Lab is interested in various projects studying the function and biogenesis of non-coding

RNA Ribonucleic acid (RNA) is a polymeric molecule essential in various biological roles in coding, decoding, regulation and expression of genes. RNA and deoxyribonucleic acid ( DNA) are nucleic acids. Along with lipids, proteins, and carbohydra ...

and the proteins that interact with RNA. A primary research goal of the lab is to elucidate the function of

Survival of Motor Neuron Survival of motor neuron or survival motor neuron (SMN) is a protein that in humans is encoded by the ''SMN1'' and ''SMN2'' genes. SMN is found in the cytoplasm of all animal cells and also in the nuclear gems. It functions in transcriptional reg ...

protein, SMN, which assembles a heptameric ring of Sm proteins on U

snRNA Small nuclear RNA (snRNA) is a class of small RNA molecules that are found within the splicing speckles and Cajal bodies of the cell nucleus in eukaryotic cells. The length of an average snRNA is approximately 150 nucleotides. They are transcribed ...

s to form

snRNP snRNPs (pronounced "snurps"), or small nuclear ribonucleoproteins, are RNA-protein complexes that combine with unmodified pre-mRNA and various other proteins to form a spliceosome, a large RNA-protein molecular complex upon which splicing of pre- ...

s that are essential components of the splicesome. Moreover, loss of functional SMN is directly linked to

spinal muscular atrophy Spinal muscular atrophy (SMA) is a rare neuromuscular disorder that results in the loss of motor neurons and progressive muscle wasting. It is usually diagnosed in infancy or early childhood and if left untreated it is the most common genetic ...

, a debilitating neurodegenerative disease that is characterize by the eventual death of motor neurons and muscular wasting. The Dreyfuss Lab is conducting research to understand the role of SMN in SMA pathology and using

high throughput screening High-throughput screening (HTS) is a method for scientific experimentation especially used in drug discovery and relevant to the fields of biology, materials science and chemistry. Using robotics, data processing/control software, liquid handling ...

to discover potential therapeutics. The lab also studies the dynamic mechanism of

RNA splicing RNA splicing is a process in molecular biology where a newly-made precursor messenger RNA (pre-mRNA) transcript is transformed into a mature messenger RNA (mRNA). It works by removing all the introns (non-coding regions of RNA) and ''splicing'' b ...

, the

RNA-binding protein RNA-binding proteins (often abbreviated as RBPs) are proteins that bind to the double or single stranded RNA in cells and participate in forming ribonucleoprotein complexes. RBPs contain various structural motifs, such as RNA recognition motif ( ...

s that determine exonic specificity, and snRNAs, that are important regulators of splicing and

mRNA In molecular biology, messenger ribonucleic acid (mRNA) is a single-stranded molecule of RNA that corresponds to the genetic sequence of a gene, and is read by a ribosome in the process of Protein biosynthesis, synthesizing a protein. mRNA is ...

maturation.

Genes or Gene Functions Discovered

eIF4A3 Eukaryotic initiation factor 4A-III is a protein that in humans is encoded by the ''EIF4A3'' gene. Function This gene encodes a member of the DEAD box protein family. DEAD box proteins, characterized by the conserved motif Asp-Glu-Ala-Asp (DEA ...

FXR1 Fragile X mental retardation syndrome-related protein 1 is a protein that in humans is encoded by the ''FXR1'' gene. The protein encoded by this gene is an RNA binding protein that interacts with the functionally similar proteins FMR1 and FXR2. Th ...

FXR2 Fragile X mental retardation syndrome-related protein 2 is a protein that in humans is encoded by the ''FXR2'' gene. Function The protein encoded by this gene is an RNA binding protein containing two KH domains and one RCG box, which is simila ...

* Gemin2 (Previously known as ''SIP1'') * Gemin3/DDX20 * Gemin4 * Gemin5 * Gemin6 * Gemin7 * hnRNPA1 * RA33 * hnRNPC *

PTBP1 Polypyrimidine tract-binding protein 1 is a protein that in humans is encoded by the ''PTBP1'' gene. This gene belongs to the subfamily of ubiquitously expressed heterogeneous nuclear ribonucleoproteins (hnRNPs). The hnRNPs are RNA-binding protei ...

* hnRNPK * hnRNPM * hnRNPR * hnRNPU * hnRNPQ * Y14 * Magoh

References

External links

BMB: Gideon DreyfussUniversity of Pennsylvania CAMB: Gideon DreyfussDreyfuss Lab Website
{{DEFAULTSORT:Dreyfuss, Gideon 21st-century American biochemists Harvard Graduate School of Arts and Sciences alumni Fellows of the American Academy of Arts and Sciences Howard Hughes Medical Investigators Year of birth missing (living people) Living people Members of the United States National Academy of Sciences Hebrew University of Jerusalem alumni Massachusetts Institute of Technology alumni

Research

Genes or Gene Functions Discovered

See also

References

External links