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Anti-Dyskerin Antibody

品牌：Antibodies

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規(guī)格：50μl

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Anti-Dyskerin Antibody

商品詳情參考文獻相關(guān)資料

Overview

Name:	Anti-Dyskerin Antibody See all Dyskerin primary antibodies
Description:	Rabbit polyclonal antibody to Dyskerin
Specificity:	Dyskerin pAb detects endogenous levels of Dyskerin protein.
Applications:	WB, IHC, IF
Reactivity:	Human, Mouse, Rat
Immunogen:	Recombinant full length Human Dyskerin.
Host:	Rabbit
Clonality:	Polyclonal
Conjugate:	Unconjugated
Molecular Weight:	~ 57 kDa
Purity:	The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Product Form:	1mg/ml in PBS?with?0.1%?Sodium?Azide,?50%?Glycerol.

Target

Function:	Isoform 1: Required for ribosome biogenesis and telomere maintenance. Probable catalytic subunit of H/ACA small nucleolar ribonucleoprotein (H/ACA snoRNP) complex, which catalyzes pseudouridylation of rRNA. This involves the isomerization of uridine such that the ribose is subsequently attached to C5, instead of the normal N1. Each rRNA can contain up to 100 pseudouridine ('psi') residues, which may serve to stabilize the conformation of rRNAs. Also required for correct processing or intranuclear trafficking of TERC, the RNA component of the telomerase reverse transcriptase (TERT) holoenzyme.
Tissue Specificity:	Ubiquitously expressed.
Involvement in Disease:	Dyskeratosis congenita, X-linked: A rare, progressive bone marrow failure syndrome characterized by the triad of reticulated skin hyperpigmentation, nail dystrophy, and mucosal leukoplakia. Early mortality is often associated with bone marrow failure, infections, fatal pulmonary complications, or malignancy. Hoyeraal-Hreidarsson syndrome: A clinically severe variant of dyskeratosis congenita that is characterized by multisystem involvement, early onset in utero, and often results in death in childhood. Affected individuals show intrauterine growth retardation, microcephaly, cerebellar hypoplasia, delayed development, and bone marrow failure resulting in immunodeficiency.
Sequence Similarities:	Belongs to the pseudouridine synthase TruB family.
Cellular Location:	Nucleus > Nucleolus. Nucleus > Cajal body. Also localized to Cajal bodies (coiled bodies).
Database Links:	Entrez Gene: 1736?Human Entrez Gene: 245474?Mouse Entrez Gene: 170944?Rat Omim: 300126?Human SwissProt: O60832?Human SwissProt: Q9ESX5?Mouse SwissProt: P40615?Rat Unigene: 4747?Human Unigene: 291062?Mouse Unigene: 4223?Rat
Synonyms:	CBF5 Antibody CBF5 homolog Antibody Cbf5p homolog Antibody DKC Antibody DKC 1 Antibody DKC1 Antibody DKC1_HUMAN Antibody DKCX Antibody Dyskeratosis congenita 1 Antibody Dyskeratosis congenita 1 dyskerin Antibody Dyskerin Antibody H/ACA ribonucleoprotein complex subunit 4 Antibody NAP 57 Antibody NAP57 Antibody NOLA 4 Antibody NOLA4 Antibody Nopp140 associated protein of 57 kDa Antibody Nopp140-associated protein of 57 kDa Antibody Nucleolar protein family A member 4 Antibody Nucleolar protein NAP57 Antibody snoRNP protein DKC1 Antibody XAP 101 Antibody XAP101 Antibody
Information:	Target information shown above is from the UniProt Consortium.