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Home / Primary Antibodies / IHC Antibodies

Anti-DKC1 Antibody BHA11300987

SKU : BHA11300987

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Mfr.No.	BS1887
Alternative Names	H/ACA ribonucleoprotein complex subunit 4 ; CBF5 homolog ; Dyskerin ; Nopp140-associated protein of 57 kDa ; Nucleolar protein NAP57 ; Nucleolar protein family A member 4 ; snoRNP protein DKC1 ; DKC1 ; NOLA4
Antigen	DKC1
Categories	Primary Antibodies
Chromosome Location	Xq28
Clonality	polyclonal
Description	Dyskerin (NAP57) associates with the chaperone protein Nopp140 and forms a small ribonucleoprotein particle with GAR1 (NOLA1), NHP2 (NOLA2) and Nop10 for the isomerization of uridine to pseudouridine. GAR1, NHP2 and Dyskerin localize to the dense fibrillar component of the nucleolus and in nuclear Cajal bodies. The Dyskerin gene maps to chromosome Xq28. Missense mutations in the Dyskerin gene interfere with normal nuclear localization of Dyskerin and cause Dyskeratosis congenita (DKC). DKC is a rare, X-linked bone marrow disorder characterized by cutaneous hyperpigmentation, dystrophy of the nails, atrophy of the testicles and leukoplakia of the oral mucosa. The GAR1 gene maps to chromosome 4q25 and encodes a 28 kDa protein. The NHP2 gene maps to chromosome 5q35.3 and encodes a 155 amino acid protein
Host	Rabbit
Immunogen	Synthetic peptide, corresponding to amino acids 171-220 of Human Dyskerin.
Reactivity	Human, Mouse, Rat
Recombinant	FALSE
Regulatory	RUO
Species	Homo Sapiens (Human)

Swissprot	O60832
Uniprot	O60832
Weight	57 kDa

Description	Product: 1 mg/ml in Phosphate buffered saline (PBS) with 0.05% sodium azide, approx. pH 7.2. Specificity: Dyskerin (K203) polyclonal antibody detects endogenous levels of Dyskerin protein.
Purity	The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Storage	Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Applications

IHC, IF

Description

IHC ; IF

Dilution

IHC	1:50-1:200
IF	1:50-1:200

Datasheet

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SKU	Supplier No.	Size	Your price	Quantity
BHA11300987	BS1887	50µl	$190.44		Add to Cart
BHA11300987	BS1887	100µl	$294.44		Add to Cart

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