Rabbit Anti-MPDU1/FITC Conjugated antibody

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CDGIF; LEC35; mannose P dolichol utilization defect 1; Mannose-P-dolichol utilization defect 1 protein; MPDU1; MPU1_HUMAN; PQLC5; SL15; Suppressor of Lec15 and Lec35 glycosylation mutation homolog.

Cat:

SL17734R-FITC

Species Reactivity：

(predicted: Human,Mouse,Rat,Dog,Cow,Horse,Sheep,)

Immunogen：

KLH conjugated synthetic peptide derived from human MPDU1

Format：

Lyophilized or Liquid

Storage instructions：

Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

26kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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background:
This gene encodes an endoplasmic reticulum membrane protein that is required for utilization of the mannose donor mannose-P-dolichol in the synthesis of lipid-linked oligosaccharides and glycosylphosphatidylinositols. Mutations in this gene result in congenital disorder of glycosylation type If. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Dec 2008]

Function:
Required for normal utilization of mannose-dolichol phosphate (Dol-P-Man) in the synthesis of N-linked and O-linked oligosaccharides and GPI anchors.

Subcellular Location:
Membrane.

DISEASE:
Defects in MPDU1 are the cause of congenital disorder of glycosylation type 1F (CDG1F) [MIM:60936]. CDGs are a family of severe inherited diseases caused by a defect in protein N-glycosylation. They are characterized by under-glycosylated serum proteins. These multisystem disorders present with a wide variety of clinical features, such as disorders of the nervous system development, psychomotor retardation, dysmorphic features, hypotonia, coagulation disorders, and immunodeficiency. The broad spectrum of features reflects the critical role of N-glycoproteins during embryonic development, differentiation, and maintenance of cell functions.

Similarity:
Belongs to the MPDU1 (TC 2.A.43.3) family.
Contains 2 PQ-loop domains.

Database links:

Entrez Gene: 9526 Human

Omim: 604041 Human

SwissProt: O75352 Human

Unigene: 246381 Human

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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