Cell Signaling Dyskerin (D6n4k) Rabbit mAb

Name: Cell Signaling Dyskerin (D6n4k) Rabbit mAb
SKU: CSSPQ-53234S
Price: 351.00 USD

List Price $351.00 Your Price $351.00

Cell Signaling Dyskerin (D6n4k) Rabbit mAb - CSSPQ (Additional S&H or Hazmat Fees May Apply)

NETA PART: CSSPQ-53234S

MFG.PART: 53234S

UNSPSC: 12352203

Manufacturer: Cell Signaling

Size	100 µl
Reactivity	H M Mk
Sensitivity	Endogenous
Molecular Weight (kDa)	60
Source/Isotype	Rabbit IgG
Application/Dilution	{Western Blotting: 1:1000}
Storage	Supplied in 10 mm sodium HEPES (pH 7.5), 150 mm NaCl, 100 µg/mL BSA, 50% glycerol and less than 0.02% sodium azide. Store at –20°C. Do not aliquot the antibody.
Specificity/Sensitivity	Dyskerin (D6N4K) Rabbit mAb recognizes endogenous levels of total dyskerin protein.
Species Reactivity	Human, Mouse, Monkey
Source/Purification	Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Phe91 of human dyskerin protein.
Background	H box/ACA-motif small nucleolar RNAs (snoRNAs) guide snoRNA proteins (snoRNPs) to uridine residues on rRNA for the conversion to pseudouridine (1). These H/ACA snoRNPs consist of four highly conserved proteins including GAR1, NHP2, NOP10, and the catalytic component dyskerin (1-3). The core snoRNPs also bind to mammalian telomerase RNA, which contains a H/ACA-like motif in the 3’ domain. This binding results in the maintenance of telomerase levels and activity (4). Defects in the snoRNPs can lead to dyskeratosis congenita, a rare, x-linked disorder characterized by a failure of the bone marrow and an increased tumor risk (5,6). Mutations in the dyskerin gene can cause defects in translation of mRNAs containing internal ribosome entry sites (IRESs), which include mRNAs to tumor suppressors p27 and p53 and anti-apoptotic factors Bcl-xL and XIAP (7).