This document discusses protein folding and variants of the PGC-1alpha protein associated with neurodegenerative diseases. It describes how proteins fold into specific structures to perform their functions, and that the RfaH protein can fold in two ways to fulfill different roles. It was also discovered that previously unknown variants of the PGC-1alpha protein expressed specifically in the brain are associated with Huntington's disease and other neurodegenerative conditions. Studying these variants may help guide research toward finding cures for such diseases.

1. PROTEIN UNFOLDS AND
REFOLDS FOR NEW FUNCTION
AND
BRAIN VARIANTS OF PROTEIN
ASSOCIATED WITH HUNTINGTON'S AND
OTHER NEURODEGENERATIVE
DISEASES IDENTIFIED
By
Maria Alejandra Lugo Posada
Medical Student
27/08/12

4. INTRODUCTION
The gene translation is the step of the information carried by the RNAm to protein; The
last step is traducction embodying the information contained in the DNA, by decoding
order that finally determines the sequence of specific polypeptide chains.
There are some differences between protein synthesis in eukaryotic and prokaryotic
cells: In eukaryotic protein synthesis, it is usually the cytoplasmatic ribosomes that
translate nuclear genes. the ribosomes of eukaryotic cells are larger and contain more
rRNA and protein molecules than those of prokaryotes. in addition, eukaryotes have
more initiation factors and a more complex initiation procedure.
The proteins are active mediators in most cellular processes, carrying out the functions
determined by the information encoded in the DNA.
adequate protein synthesis is of great importance, since large quantities of diseases are
associated with abnormal synthesis these.
Here we will see some news about the proper role of the proteins for their proper
folding, and consequences of polymorphisms.

5. PROTEIN UNFOLDS AND
REFOLDS FOR NEW
FUNCTION
So that should work correctly folded proteins in a right way,
and there are certain folds that are more favorable than
others.
In protein folding are two common types: and are the alpha
and beta structure:
•alpha structure in the molecule is coiled in a helix.
•beta structure in the hydrogen bonds are formed between
different parts of the chain more or less parallel to each
other. and going in the same direction and in the opposite
direction.

6. DNA directed RNA synthesis
RNA directs protein synthesis
Protein folding
•Catalyze chemical reactions
Protein performs its functions: •Transmits information between
specific cells
•Participates in the immune
system
•CONTROLS GENE EXPRESSION

7. Shows that RfaH is even more effective as a
translation factor -- about 100 times more
powerful than it is as a transcription factor.
Because RfaH has more power during translation
because it recruits the ribosome molecule under
circumstances when there isn't enough
information in the mRNA for the ribosome to do
its protein construction.

8. Normally the
ribosome binds to a
specific sequence. If
there is no
sequence, it doesn't
know where to bind.
Is believed that the
ribosome recognizes
RfaH can interact
RfaH instead of this
with the ribosome
sequence and starts
once it refolds.
translation.
it can bind to both
the RNA polymerase
and the ribosome at
the same time and
link them together

9. OBSERVATION
An extremely important step in gene translation is the
folding of the protein, since the level agree having
structure and shape as the fold meet or no function, as
is the case RfaH protein that can fulfill two different
functions when folded in two ways different.
we know that proteins are very large and complex
molecules, and each amino acid that composes this at a
specific site, agree to the information of DNA, which
gives the protein necessary for one structure what role
(primary structure, secondary, tertiary and quaternary).

10. BRAIN VARIANTS OF PROTEIN ASSOCIATED
WITH HUNTINGTON’S AND OTHER
NEURODEGENERATIVES DISEASES
IDENTIFIED
Was discovered a large new region in
the genetic code of the protein PGC-
1alpha. Previously unknown variations
of the protein, which can be found
specifically in the brain, are produced
from this region

11. .
A PROMOTER:
Is a DNA segmente usually occurring upstream
from a gene that can ultimately control how that
gene is expressed as a protein. The transmission of
genetic information from DNA to RNA
molecules, i.e. transcription, is an important
intermediate step in this process.

12. Was demonstrated that these
previously unknown transcripts
are produced specifically in
human brain cells and are at
least as common there as the
reference transcripts.

13. NEURODEGENERATIVE DISEASES
Are diseases without Preferred systemic Are
evidence of Prevalence
involvement of one progressive
exogenous injury increases with
or several neural character.
(infection, ischemia, age.
systems (substantia
trauma, etc.). nigra, hippocampus,
etc)
The cause of neurodegenerative
diseases is often unknown, but is
close relationship with l protein
variation, such as of the PGC-1alpha
protein.

14. OBSERVATION
Neurodegenerative diseases such as Huntington's disease, Parkinson's
and Alzheimer's, are diseases affecting much of the population, and are
diseases that have no cure, only methods found that prevented me to
progress so fast, for it is great importance the investigation of causes of
these diseases, in order to guide research in a better way. The discovery
of the variation in the PGC-1alpha protein, provides a way of research.

15. MEDICAL UTILITY
PROTEIN UNFOLDS AND
REFOLDS FOR NEW FUNCTION
The results about the folding of the
protein have significant implications for
studies of gene expression control and
protein structure.

16. MEDICAL UTILITY
PROTEIN UNFOLDS AND
REFOLDS FOR NEW FUNCTION
In some cases is inefficient translation and in such
cases proteins are needed to help in this process, as
is the case Genes that control bacterial virulence are
famous for being designed in a way that makes their
translation very inefficient -- hence, they need
specialized proteins to help their expression

17. MEDICAL UTILITY
BRAIN VARIANTS OF PROTEIN ASSOCIATED WITH HUNTINGTON’S AND
OTHER NEURODEGENERATIVES DISEASES IDENTIFIED
The protein PGC-1 alpha is a protein with many essential functions in our
body, but is also associated with diseases neurodegenerative wherefore it
is of great clinical importance because in a way that guides the study for
the cure of these diseases.

18. MEDICAL UTILITY
BRAIN VARIANTS OF PROTEIN ASSOCIATED WITH HUNTINGTON’S AND
OTHER NEURODEGENERATIVES DISEASES IDENTIFIED
The promoter is a control element, which is recognized
by the RNA polymerase to begin transcription, and found
a new promoter that is active in the nervous system, and
which interferes with transcription to the gene coding for
protein PGC-1 alpha, so we see that in this study of
neurodegenerative diseases is also of great importance
to study the promoters.

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