WordNet

(meteorology) a unit of pressure equal to a million dynes per square centimeter; "unfortunately some writers have used bar for one dyne per square centimeter"
secure with, or as if with, bars; "He barred the door"
(law) a railing that encloses the part of the courtroom where the judges and lawyers sit and the case is tried; "spectators were not allowed past the bar"
a counter where you can obtain food or drink; "he bought a hot dog and a coke at the bar"
a heating element in an electric fire; "an electric fire with three bars"
a horizontal rod that serves as a support for gymnasts as they perform exercises
a rigid piece of metal or wood; usually used as a fastening or obstruction or weapon; "there were bars in the windows to prevent escape"
an obstruction (usually metal) placed at the top of a goal; "it was an excellent kick but the ball hit the bar"
a submerged (or partly submerged) ridge in a river or along a shore; "the boat ran aground on a submerged bar in the river"
prevent from entering; keep out; "He was barred from membership in the club" (同)debar, exclude
lay bare; "bare your breasts"; "bare your feelings"
completely unclothed; "bare bodies"; "naked from the waist up"; "a nude model" (同)au naturel, naked, nude
providing no shelter or sustenance; "bare rocky hills"; "barren lands"; "the bleak treeless regions of the high Andes"; "the desolate surface of the moon"; "a stark landscape" (同)barren, bleak, desolate, stark
just barely adequate or within a lower limit; "a bare majority"; "a marginal victory" (同)marginal
apart from anything else; without additions or modifications; "only the bare facts"; "shocked by the mere idea"; "the simple passage of time was enough"; "the simple truth" (同)mere, simple
lacking in amplitude or quantity; "a bare livelihood"; "a scanty harvest"; "a spare diet" (同)scanty, spare
having everything extraneous removed including contents; "the bare walls"; "the cupboard was bare" (同)stripped
lacking a surface finish such as paint; "bare wood"; "unfinished furniture" (同)unfinished
lacking its natural or customary covering; "a bare hill"; "bare feet"
a pattern of symptoms indicative of some disease
a complex of concurrent things; "every word has a syndrome of meanings"
an agranulocytic leukocyte that normally makes up a quarter of the white blood cell count but increases in the presence of infection (同)lymph cell

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(木または金属の)『棒』;棒状の物 / 横木,(戸・とびらなどの)かんぬき,横さん / (一般に)(…対する)『障害』《+『to』(『against』)+『名』》 / (また『sand bar』)(河口・港口の)砂州 / (光・色などの)線条,しま,帯 / (酒場・旅館などの)酒を出すカウンター;バー,酒場;簡易食堂 / 《the~》(法廷の裁判官席・被告席・弁護士席と一般席との間の)仕切り;《比諭(ひゆ)的に》法廷 / 《集合的に》弁護士団,法曹界;弁護士[業]: / (楽譜の小節を分ける)縦線;小節 / 〈戸・門など〉‘に'『かんぬきをさす』 / 〈道・通行など〉'を'『妨げる』,はばむ / 《通例受動態で》…‘に'線(筋)をつける / …'を'禁じる(forbid) / (…から)…'を'除外する,締め出す《+『名』+『from』(『out of』)+『名』》;(…に)…'を'とじ込める《+『名』+『in』+『名』》: / …の除いて(except)
『裸の』,『おおいのない』;露出した,むき出した / 『からの』,なにもない(empty) / 『ありのままの』,赤裸々な / 《名詞の前にのみ用いて》『かろうじての』,やっとの;ただ…だけの(mere) / …'を'裸にする,あらわにする;〈刀〉'を'燃く
(疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2012/12/19 00:15:24」(JST)

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Bare lymphocyte syndrome is a condition caused by mutations in certain genes of the major histocompatibility complex. It is a form of severe combined immunodeficiency.^[1]

Classification

Type 1: MHC class I
Type 2: MHC class II

Presentation

The bare lymphocyte syndrome, type II (BLS II) is a rare recessive genetic condition in which a group of genes called major histocompatibility complex class II (MHC class II) are not expressed.

The result is that the immune system is severely compromised and cannot effectively fight infection. Clinically, this is similar to severe combined immunodeficiency (SCID), in which lymphocyte precursor cells are improperly formed.

Diarrhea can be among the associated conditions.^[2]

Genetics

BLS II

The genetic basis for BLSII is not due to defects in the MHC II genes themselves. The genetic basis is the result of mutations in genes that code for proteins (transcription factors) that normally regulate the expression (gene transcription) of the MHC II genes. That is, one of the several proteins that are required to switch on MHC II genes in various cells types (primarily those in the immune system) is absent. The genes responsible were cloned by the laboratories of Bernard Mach^[3] in Switzerland and Jeremy Boss^[4] at Emory University in Atlanta, Georgia.

Mutation in any one of four genes can lead to BLS II. The genes' names are:

class II trans-activator (CIITA)
regulatory factor of the X box 5 (RFX5)
RFX-associated protein (RFXAP)
RFX ankyrin repeats (RFXANK; also known as RFXB)

BLS I

BLS I, also called "HLA class I deficiency", which is much more rare, is associated with TAP2, TAP1, or TAPBP.^[5]

Treatment

Though BLSII is an attractive candidate for gene therapy, bone marrow transplant is currently the only treatment.

References

^ DeSandro AM, Nagarajan UM, Boss JM (September 2000). "Associations and interactions between bare lymphocyte syndrome factors". Mol. Cell. Biol. 20 (17): 6587–99. doi:10.1128/MCB.20.17.6587-6599.2000. PMC 86141. PMID 10938133. http://mcb.asm.org/cgi/pmidlookup?view=long&pmid=10938133.
^ "Immunologic Disease and Disorders". http://www.lib.mcg.edu/edu/esimmuno/casehist/diseases/barelymp.htm.
^ Reith W, Mach B (2001). "The bare lymphocyte syndrome and the regulation of MHC expression". Annu. Rev. Immunol. 19: 331–73. doi:10.1146/annurev.immunol.19.1.331. PMID 11244040.
^ DeSandro A, Nagarajan UM, Boss JM (1999). "The bare lymphocyte syndrome: molecular clues to the transcriptional regulation of major histocompatibility complex class II genes". Am. J. Hum. Genet. 65 (2): 279–86. doi:10.1086/302519. PMC 1377925. PMID 10417269. //www.ncbi.nlm.nih.gov/pmc/articles/PMC1377925/.
^ Online 'Mendelian Inheritance in Man' (OMIM) 604571

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UpToDate Contents

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1. 複合免疫不全 combined immunodeficiencies
2. Sézary症候群の臨床症状、病理学的特徴、および診断 clinical presentation pathologic features and diagnosis of sezary syndrome
3. 主要組織適合性複合体（MHC）の構造および機能 major histocompatibility complex mhc structure and function
4. リンパ球増加症またはリンパ球減少症を有する患者へのアプローチ approach to the patient with lymphocytosis or lymphocytopenia
5. Palmoplantar pustulosis: Epidemiology, clinical features, and diagnosis

English Journal

3,3'-diindolylmethane enhances taxotere-induced growth inhibition of breast cancer cells through downregulation of FoxM1.

Ahmad A, Ali S, Wang Z, Ali AS, Sethi S, Sakr WA, Raz A, Rahman KW.SourceDepartment of Pathology, Karmanos Cancer Institute, Wayne State University School of Medicine, Detroit, MI.
International journal of cancer. Journal international du cancer.Int J Cancer.2011 Oct 1;129(7):1781-91. doi: 10.1002/ijc.25839. Epub 2011 Mar 8.
Emerging evidence suggests that the transcription factor Forkhead Box M1 (FoxM1) is associated with aggressive human carcinomas, including breast cancer. Because elevated expression of FoxM1 has been observed in human breast cancers, FoxM1 has attracted much attention in recent years as a potential
PMID 21154750