Maturity enzyme that lead to insufficient insulin release

Maturity
Onset Diabetes of the Young (MODY), is a kind of diabetes that caused by genes ,
monogenic form of diabetes. This gene is dominant mode of inheritance. We have
kinds of mutations that affects the enzyme that lead to insufficient insulin
release from pancreatic cells.

 

v MODY
types and treatment:

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                The mutation in the gene can be
in:

o  
pancreatic development

o  
insulin gene expression

o  
glucose sensing

MODY is a
type of diabetes that occurs in children and young adults.(1)

 

v The
most common types of MODY are:

·        
HNF1-alpha : 70 % of cases of MODY. This kind
causes diabetes by lowering the amount of insulin made by the pancreas.

·        
HNF4-alpha : This type is not common as the
previous. People with this gene mutation are around 4kg when given birth.

·        
HNF1-beta : This kind causes variety of
problems like renal cysts ,uterine abnormalities and gout, as well as diabetes.

·        
Glucokinase : mutation in this gene will make
the level of blood glucose to be higher than it should be.

 

Ø  MODY should
be diagnosing early for:

o  
To take the right kind of treatment.

o  
There is a chance by 50% that
parents will pass MODY to their child.

o  
Genetic testing can be offered to
other family members.

 Because we have 6 types of mutations, the
mutation that is caused in one family can be different from the mutation that
is caused in another family.(2)

 

 

MODY affects
approximately 1-2% of people with diabetes. Some cases was not recognized. The main
properties of MODY:

o  
Diabetes occurs at age before 25.

o  
Diabetes is inherited from
generation to another.

o  
It can be treated by diet or medication
and sometimes insulin.(3)

                                                                                                      

The
diagnosis of MODY began based on presence of non-ketotic hyperglycemia in young
adults and the relation to family history of diabetes. New tests has shown that
MODY can occur at any age ,also in some cases does not come from inheritance. (4)

MODY 2 and
MODY 3 are the most common types.

New updates
found that mutations in the HNF1B (MODY 5) is related to:

o  
pancreatic agenesis

o  
renal abnormalities

o  
genital tract malformations

o  
liver dysfunction.

When
comparing the 6 types, we will find that MODY 1, 2, 3, and 5 are the most
common. The rest subtypes have a much lower prevalence. (5)

 

v
Treatment:

Treatment
for MODY depends on the type of mutation and also depends if the it is diabetes
type 1 or 2.

Treatment
ranges from diet and exercises and ends with taking insulin.

MODY 2 is a
simply treated MODY because if diagnosed early can usually be managed by eating
right and exercising regularly.

MODY 1, 3,
and 4 can usually be managed with a type of medicine called sulfonylurea
therapy.

MODY 5 needs
many treatments because it is a cause of medical problems not just for the
blood sugar level. The genes for MODY 7 were recently discovered.(6)

v
Conclusion:

We can
conclude from all the above hat MODY is a common cause of monogenic diabetes and
it is approximately 1% to 2% of all diabetes cases.

MODY prevalence
is low and its identification of MODY genes has implications in diabetes
pathogenesis. Genetic heterogeneity is the main clinical characteristics of
MODY. Every subtype has a treatment ranges from diet to insulin.

MODY should
be diagnosed early before it can make damages, especially young people has it.
Patients and their families should take care should be provided individualized
treatment  based on the subtype, and
prognosis predictions.

Diagnosing MODY is a major problem and needs a
lot of work and it is a hard job.