Cell Biology Antibodies 15

Anti-CHST14 Antibody (CAB18527)

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SKU:
CAB18527
Product Type:
Antibody
Reactivity:
Human
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Cell Biology

Description

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Antibody Name:Anti-CHST14 Antibody
Antibody SKU:CAB18527
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human
Host Species:Rabbit
Immunogen:Recombinant protein of human CHST14.
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human
Positive Samples:
Immunogen:Recombinant protein of human CHST14.
Purification Method:Affinity purification
Storage Buffer:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:Email for sequence
Gene ID:113189
Uniprot:Q8NCH0
Cellular Location:
Calculated MW:
Observed MW:Refer to figures
Synonyms:ATCS, D4ST1, EDSMC1, HNK1ST, CHST14
Background:
UniProt Protein Function:CHST14: Catalyzes the transfer of sulfate to position 4 of the N-acetylgalactosamine (GalNAc) residue of dermatan sulfate. Plays a pivotal role in the formation of 4-0-sulfated IdoA blocks in dermatan sulfate. Transfers sulfate to the C-4 hydroxyl of beta1,4-linked GalNAc that is substituted with an alpha-linked iduronic acid (IdoUA) at the C-3 hydroxyl. Transfers sulfate more efficiently to GalNAc residues in -IdoUA-GalNAc-IdoUA- than in -GlcUA-GalNAc-GlcUA-sequences. Has preference for partially desulfated dermatan sulfate. Addition of sulfate to GalNAc may occur immediately after epimerization of GlcUA to IdoUA. GlcUA to IdoUA. Appears to have an important role in the formation of the cerbellar neural network during postnatal brain development. Defects in CHST14 are the cause of Ehlers-Danlos syndrome musculocontractural type (EDSMC). It is a form of Ehlers-Danlos syndrome characterized by distinctive craniofacial dysmorphism, congenital contractures of thumbs and fingers, clubfeet, severe kyphoscoliosis, muscular hypotonia, hyperextensible thin skin with easy bruisability and atrophic scarring, wrinkled palms, joint hypermobility, and ocular involvement. Belongs to the sulfotransferase 2 family.
UniProt Protein Details:

Protein type:Membrane protein, integral; Glycan Metabolism - chondroitin sulfate biosynthesis; Transferase; EC 2.8.2.35

Chromosomal Location of Human Ortholog: 15q15.1

Cellular Component: Golgi membrane

Molecular Function:N-acetylgalactosamine 4-O-sulfotransferase activity

Biological Process: dermatan sulfate biosynthetic process; dermatan sulfate proteoglycan metabolic process

Disease: Ehlers-danlos Syndrome, Musculocontractural Type 1

NCBI Summary:This gene encodes a member of the HNK-1 family of sulfotransferases. The encoded protein transfers sulfate to the C-4 hydroxyl of N-acetylgalactosamine residues in dermatan sulfate. Mutations in this gene have been associated with adducted thumb-clubfoot syndrome.[provided by RefSeq, Mar 2010]
UniProt Code:Q8NCH0
NCBI GenInfo Identifier:61211839
NCBI Gene ID:113189
NCBI Accession:Q8NCH0.2
UniProt Secondary Accession:Q8NCH0,Q6PJ31, Q6UXA0, Q96P94,
UniProt Related Accession:Q8NCH0
Molecular Weight:42,997 Da
NCBI Full Name:Carbohydrate sulfotransferase 14
NCBI Synonym Full Names:carbohydrate sulfotransferase 14
NCBI Official Symbol:CHST14  
NCBI Official Synonym Symbols:ATCS; D4ST1; EDSMC1; HNK1ST  
NCBI Protein Information:carbohydrate sulfotransferase 14
UniProt Protein Name:Carbohydrate sulfotransferase 14
UniProt Synonym Protein Names:Dermatan 4-sulfotransferase 1; D4ST-1; hD4ST1
Protein Family:Carbohydrate sulfotransferase
UniProt Gene Name:CHST14  
UniProt Entry Name:CHSTE_HUMAN
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