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Eight patients had features of KCS while four had xerostomia. Paper-2841799.
We have designated this new motif as KCS, for kinase conserved sequence. Paper-869664.
The autosomal recessive Kenny-Caffey syndrome locus maps to chromosome 1q42-q43. Paper-1672946.
Sialochemistry was used to detect salivary gland involvement in KCS patients. Paper-3137849.
We present a 12-year-old girl with Kenny-Caffey syndrome and bilateral optic atrophy. Paper-7334823.
Tracheobronchiomegaly. The Mounier-Kuhn syndrome in a patient with the Kenny-Caffey syndrome. Paper-7439409.
Clinical symptoms of KCS may be more dependent on T-cell activation and resultant inflammation than previously believed. Paper-9523795.
Millions of individuals suffer from a health condition known as keratoconjunctivitis sicca ( KCS, also known as 'dry eye'). Paper-11424334.
Kenny-Caffey syndrome ( KCS) is an osteosclerotic bone dysplasia with associated hypocalcemia and ocular abnormalities. Paper-1672946.
A maximum multipoint LOD score of 14.32 was obtained at marker D1S2649, confirming linkage of SSS to the same region as autosomal recessive KCS. Paper-1905306.
SSS resembles the autosomal recessive form of Kenny-Caffey syndrome ( KCS; MIM244460), with similar manifestations but lacking osteosclerosis. Paper-1905306.
Protein binding to the KCS element is not dependent upon IFN treatment and correlates with transcriptional activity of the PKR promoter. Paper-10397530.
Hypoparathyroidism, retarded growth and development, and dysmorphism or Sanjad-Sakati syndrome: an Arab disease reminiscent of Kenny-Caffey syndrome. Paper-2117729.
Site-directed mutagenesis was performed, whereby every base pair position within the KCS element was replaced by each of the other three alternatives. Paper-1630187.
METHODS: Assessment of 210 people, 130 with non-Sjogren's keratoconjunctivitis sicca (non-SS KCS), 32 with Sjogren's Syndrome (SS), and 48 control subjects. Paper-11029347.
We have carried out a systematic analysis of the 5' flanking region of the human Pkr gene to define how the novel KCS element acts to affect basal as well as IFN-inducible transcription. Paper-1751392.
Using eight consanguineous Kuwaiti kindreds, a genome-wide search for linkage to the gene causing the autosomal recessive form of KCS was performed with polymorphic short tandem repeat markers. Paper-1672946.
It is the authors' opinion that the functional visual acuity tester and the new tear stability analysis system will be widely used to improve diagnosis and evaluate treatment outcomes in KCS. Paper-12408493.
New and emerging treatment options using androgens to suppress glandular inflammation and muscarinic M3 agonists, i.e., cevimeline and pilocarpine, show promise as treatment options for SS- KCS. Paper-8560652.
Mechanism of interferon action: identification of essential positions within the novel 15-base-pair KCS element required for transcriptional activation of the RNA-dependent protein kinase pkr gene. Paper-1630187.
The strict conservation of sequence, distance, and position of KCS, relative to ISRE, together with mutagenesis results, suggest an important functional role for the newly recognized KCS motif. Paper-869664.
In addition to the assignment of the Saudi SSS and Kuwaiti KCS syndromes to overlapping genetic intervals, comparison of the haplotypes unexpectedly demonstrated that the diseases shared an identical haplotype. Paper-1905306.
The results establish 5' NNRRRGG(C,A,T)GGRGYYN 3', where R stands for purine and Y stands for pyrimidine, as the consensus sequence for the KCS element, both for basal and for IFN-inducible promoter activity. Paper-1630187.
Other than the ISRE and novel KCS elements, the overall organization of potential binding sites for transcription factors was not well conserved between the IFN-inducible promoters of the human and mouse Pkr genes. Paper-869664.
CONCLUSIONS: Treatment of dry eye syndrome for 6 months with topical CsA resulted in an increase in goblet cell numbers in patients with NSS-KCS and SS- KCS and a decrease in epithelial turnover in those with NSS-KCS. Paper-9197149.
CONCLUSIONS: The authors' findings indicate that patients with SS- KCS or NS- KCS have conjunctival inflammation manifested by inflammatory cell infiltrates and upregulation of expression in markers of immune activation. Paper-9523795.
PURPOSE: To examine the conjunctiva of patients with Sjögren's syndrome keratoconjunctivitis sicca (SS- KCS) and non-Sjögren's keratoconjunctivitis sicca (NS- KCS) for evidence of immune-based inflammation. Paper-9523795.
Competition EMSA established that constitutively expressed nuclear proteins bound the KCS element selectively; KCS protein binding activity correlated with promoter activity in the transient transfection reporter assay. Paper-1630187.
The Pkr promoter region has a novel 15-bp DNA element designated as KCS required for transcriptional activity that is located 4 bp upstream of a 13-bp IFN- stimulated response element (ISRE) that confers inducibility by type I IFN. Paper-1751392.
Fifty patients suffering from KCS and exhibiting various patterns of corneal staining with fluorescein were examined for adenovirus types 3, 7, 8, and 14, herpes simplex, and the TRIC group by tissue culture and immunofluorescein techniques. Paper-2238395.
The girls had some unusual features--microcephaly and psychomotor retardation--that distinguish the Kenny-Caffey syndrome profile in Arab children from the classical Kenny-Caffey syndrome phenotype characterized by macrocephaly and normal intelligence. Paper-8281187.
One hundred sixty patients with Sjogren's syndrome have been evaluated and managed at Scripps Clinic. Objective diagnosis has relied heavily on rose-bengal vital staining and corneal slit lamp examination to establish the presence of KCS and lip biopsy. Paper-4897864.
OBJECTIVES: To compare conjunctival goblet cell numbers as well as epithelial turnover in patients with non-Sjögren syndrome--associated keratoconjunctivitis sicca (NSS-KCS) and those with SS- KCS before and after 6 months of treatment with topical cyclosporine A (CsA) ophthalmic emulsion. Paper-9197149.
Kenny-Caffey Syndrome is a rare syndrome characterized by growth retardation, uniformly small slender long bones with medullary stenosis, thickened cortex of the long bones, hypocalcemia possibly with tetany at an early age, hyperphosphatemia, ocular abnormalities, and normal intelligence. Paper-6265219.
However, the more common manifestation seen in patients with SS is immune mediated noninfectious inflammation of the lacrimal glands and ocular surface, resulting in decreased tear production and inflammatory changes on the ocular surface known as SS associated keratoconjunctivitis sicca (SS- KCS). Paper-8560652.
METHODS: In this case-controlled study, impression cytology specimens were taken in a series of patients with vernal keratoconjunctivitis (n=21), giant papillary conjunctivitis (n=6), or keratoconjunctivitis sicca ( KCS; n=17), or receiving topical antiglaucoma treatments (n=31), and from 20 normal subjects. Paper-11043346.
RESULTS: In the past 44 years, esp. from 1979 on, in a planned way, we have systematically researched the dacryology with main results below: created new word "Leiyexue" in Chinese & its English words, dacryology & lacrimology by adding-ology to dacrya & lacrima; made LS consisted of reflex LG & LP, and basic LG besides; found out the normal & abnormal data of tear and serum LF & its iron bindingcapacity; MDH, LDH & isoenzymes; beta 2 m; MDA & antioxidants; trace elements, prostaglandins, intramuscular gentamicin passing the blood-tear barrier; bacteria, fungi, anaerobe, virus & parasite; Ig, T cell & subsets, Langerhan cell, HLA antigen; in cidence ( KCS, 0.3-3%, trancomatous DE 0.003%); the parallel correlations between DE graded by rb, BUT & Slt; and in SS, lymphocytes infiltration in LG; Lab G & conjunctiva; LF (< 100 mg%) & beta 2 m (> 1.7 mg%); and testosterone receptors in LG; negated presence of tear Beta-lysim; set up a series of models for researching & a complete set of measures to diagnose, grade, judge and treat DE, deviced new operations. Paper-11655892.
These accession numbers are used for gene KCS: .
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