Anti-CHST14 Antibody (CAB18527)
- SKU:
- CAB18527
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
Frequently bought together:
Description
抗体名: | Anti-CHST14 Antibody |
抗体コード: | CAB18527 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB |
反応性: | Human |
宿主種: | Rabbit |
免疫原: | Recombinant protein of human CHST14. |
申し込み: | WB |
推奨希釈: | WB 1:500 - 1:2000 |
反応性: | Human |
ポジティブサンプル: |
免疫原: | Recombinant protein of human CHST14. |
精製方法: | Affinity purification |
ストレージバッファ: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
アイソタイプ: | IgG |
順序: | Email for sequence |
遺伝子ID: | 113189 |
Uniprot: | Q8NCH0 |
セルラーロケーション: | |
計算された分子量: | |
観察された分子量: | Refer to figures |
同義語: | ATCS, D4ST1, EDSMC1, HNK1ST, CHST14 |
バックグラウンド: |
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 |