DISORDERS RELATED TO NUCLEAR FACTOR KAPPA B ESSENTIAL MODULATOR (NEMO) IMMUNODEFICIENCY IN CHILDREN: A REVIEW
Journal Title: International Research Journal of Pharmacy (IRJP) - Year 2012, Vol 3, Issue 5
Abstract
IKBKG is a gene that encodes for a protein which regulates nuclear factor kappa-B (NF-KB). This NF-KB not only plays an integral role in body’s inflammatory and immune responses but also affects ectodermal development. The protein complex NF-KB is present in the cytoplasm in an inactive form and requires nuclear factor kappa-B essential modulator (NEMO) for its activation. Impaired expression of NEMO, therefore, results in immunodeficiency. This article throws light on the three major disorders that occur in children due to mutations in NEMO. The disorders include ectodermal dysplasia, incontinentia pigmenti and osteopetrosis. Ectodermal dysplasia involves dystrophies of the ectodermal structures causing hypohidrosis, hypotrichosis etc. Incontinentia pigmenti mainly affects the skin but teeth, bones, brain and eyes are also not left unaffected in certain cases. Osteopetrosis targets the bones.
Authors and Affiliations
Priyanka Harbola , Pragya Mishra
Keywords
Related Articles
- EP ID EP92708
- DOI -
- Views 113
- Downloads 0
SYNTHESIS AND BIOLOGICAL STUDIES OF CHERIMOLACYCLOPEPTIDE E AND ITS N- METHYLATED ANALOG
The N-methyl derivative of Cherimolacyclopeptide E cyclo[Phe-(Dimethyl)Tyr-Pro-Gly-Leu-Gly] was synthesized using solution phase peptide synthesis. The synthesized compound was characterized by spectral data and was eval...
DEVELOPMENT AND VALIDATION OF METHOD FOR DETERMINATION OF CICLESONIDE IN (MICRONIZED) BY HPLC
A simple, selective and rapid reversed phase high performance liquid chromatographic (RPHPLC) method for the analysis of Ciclesonide has been developed and validated. The separation was achieved from HPLC Column (Waters...
OXIDATIVE STRESS INDUCED ULCER PROTECTED BY NATURAL ANTIOXIDANTS: A REVIEW
Oxidative stress is caused by an imbalance between the production of reactive oxygen and a biological system's ability to readily detoxify the reactive intermediates, which ultimately leads to oxidative deterioration of...
EFFECT OF LEPTADENIA RETICULATA LINN ON STRESS MODULATED SEXUAL BEHAVIOR OF MALE RATS
In the day today life the persons living and working in the area were the environmental factors such as chemical, physical, or emotional, stress may adversely affect the testicular functions that is enhancement of testic...
SYNTHESIS, CHARACTERIZATION AND ANTI MICROBIAL STUDIES OF AgNP’S USING PROBIOTICS
Recent researches speak about the utilization of biological systems especially enterobacteria to synthesize nanoparticles is a novel approach. The present study demonstrates an eco-friendly and low cost protocol fo...