Rabbit Anti-SLC4 Methylsterol Oxidase/FITC Conjugated antibody

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1500001G16Rik; C 4 methyl sterol; C78600; DESP4; ERG25; Methylsterol monooxygenase 1; MGC104344; MSMO1; MSMO1_HUMAN; OTTHUMP00000219757; OTTHUMP00000219758; OTTHUMP00000219759; OTTHUMP00000219760; sterol C4 methyl oxidase like.

Cat:

SL2947R-FITC

Species Reactivity：

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

Immunogen：

KLH conjugated synthetic peptide derived from human ERG25

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：

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

Host：

Rabbit

Calculated MW：

35kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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background:
Belonging to the sterol desaturase family, ERG25 is a 293 amino acid enzyme that performs the first of three enzymatic steps required to remove the two methyl groups leading to cholesterol. ERG25 is a multi-pass membrane protein that resides within the plasma membrane and the membrane of the endoplasmic reticulum. Concentrations of ERG25 mRNA increase when subjected to low iron growth conditions, suggesting that the enzyme is not regulated by iron but by an end product of the ergosterol pathway. Low-density lipoprotein (LDL) downregulates ERG25 expression in the vascular wall, an event that may be inhibited via SREBP overexpression. ERG25 is encoded by a gene located on human chromosome 4q32.3.

Function:
Catalyzes the first step in the removal of the two SLC4 methyl groups of 4,4-dimethylzymosterol.

Subcellular Location:
Endoplasmic reticulum membrane; Multi-pass membrane protein.

DISEASE:
Microcephaly, congenital cataract, and psoriasiform dermatitis (MCCPD) [MIM:616834]: An autosomal recessive inborn error of cholesterol metabolism characterized by accumulation of a large amount of methylsterols, particularly dimethylsterols, in affected individuals. Patients manifest psoriasiform dermatitis, arthralgias, congenital cataracts, microcephaly, and developmental delay. {ECO:0000269|PubMed:21285510, ECO:0000269|PubMed:24144731}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Belongs to the sterol desaturase family.

Database links:

Entrez Gene: 6307 Human

Entrez Gene: 66234 Mouse

Entrez Gene: 140910 Rat

Omim: 607545 Human

SwissProt: Q3160 Human

SwissProt: Q9CRA4 Mouse

SwissProt: O35532 Rat

Unigene: 105269 Human

Unigene: 30119 Mouse

Unigene: 7167 Rat

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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