Two novel CLN6 mutations in variant late-infantile neuronal ceroid lipofuscinosis patients of Turkish origin.

lipofuscinoses neuronal ceroid (NCLs) is the most common neurodegenerative disorder-onset period marked by autosomal recessive inheritance, epileptic seizures, progressive psychomotor damage, visual failure, and early death. At least seven subtypes of NCLs time-onset have been identified from which the final form of infantile-onset (LINCLs) is genetically most heterogeneous with four genes that underlie identified.

A variant form of LINCL (vLINCL) present in patients Turkey has been regarded as a clinical entity and different genetic (CLN7). However, we have recently demonstrated that mutations in the gene CLN8 account for a subset of Turkey vLINCL. Towards identifying genes CLN7 us here filtered NCL loci known homozygosity in a family of nine Turkish vLINCL.

This locus is issued in seven families who may represent the ‘true’ Turkish vLINCL. In two families, we identified two novel homozygous mutation in the gene CLN6: base substitution intronic (c.542 + 5G> T) affects splicing of the transcript and nonsense mutation (c.663C> G) creates a stop codon at Tyrosine 221. These data indicate that CLN6 mutations, other than those of CLN8, should be considered as a diagnostic alternative in patients Turkey vLINCL. The genetic background of the ‘true’ Turkish vLINCL, CLN7, still to be defined.

di-Sodium Dihydrogen Ethylenediaminetetraacetate Dihydrate

15130-95 500G
EUR 50.4

di-Sodium Dihydrogen Ethylenediaminetetraacetate Dihydrate

09592-45 500G
EUR 67.2

Disodium Zinc Ethylenediaminetetraacetate Tetrahydrate

D493658 100g
EUR 121

Calcium di-Sodium Ethylenediaminetetraacetate

15116-82 25G
EUR 14

di-Sodium Zinc Ethylenediaminetetraacetate

15126-52 25G
EUR 13.3

EDTA, Disodium Salt, Dihydrate, (Ethylenediaminetetraacetic Acid), 1kg

PC0706-1kg each Ask for price

EDTA, Disodium Salt, Dihydrate, (Ethylenediaminetetraacetic Acid), 500g

PC0706-500g each Ask for price

Copper(Ⅱ) di-Sodium Ethylenediaminetetraacetate

15119-52 25G
EUR 18.9

Manganese(Ⅱ) di-Sodium Ethylenediaminetetraacetate

15123-82 25G
EUR 88.2

Ethylenediaminetetraacetic acid disodium salt dihydrate Reagent Grade

E08850 100G
EUR 119.52
Description: CAS N° 139-33-3

Magnesium di-Sodium Ethylenediaminetetraacetate Tetrahydrate

15122-92 25G
EUR 15.4

di-Potassium Dihydrogen Ethylenediaminetetraacetate

15108-05 500G
EUR 81.2

di-Potassium Dihydrogen Ethylenediaminetetraacetate

15108-92 25G
EUR 12.46

0.1mol/l-di-Sodium Dihydrogen Ethylenediaminetetraacetate Solution

37310-55 500ML
EUR 18.9

0.01mol/l-di-Sodium Dihydrogen Ethylenediaminetetraacetate Solution

37311-45 500ML
EUR 18.9

0.2mol/l-di-Sodium Dihydrogen Ethylenediaminetetraacetate Solution

37336-35 500ML
EUR 25.9

0.02mol/l-di-Sodium Dihydrogen Ethylenediaminetetraacetate Solution

37337-25 500ML
EUR 19.6

0.05mol/l-di-Sodium Dihydrogen Ethylenediaminetetraacetate Solution

37341-55 500ML
EUR 19.6

tetra-Sodium Ethylenediaminetetraacetate

15114-02 25G
EUR 13.3

tetra-Sodium Ethylenediaminetetraacetate

15114-15 500G
EUR 35.7

Iron(Ⅲ) Monosodium Ethylenediaminetetraacetate

15120-12 25G
EUR 15.4

Iron(Ⅲ) Monosodium Ethylenediaminetetraacetate

15120-25 500G
EUR 48.65

tri-Sodium Hydrogen Ethylenediaminetetraacetate

15113-12 25G
EUR 10.85

tri-Sodium Hydrogen Ethylenediaminetetraacetate

15113-25 500G
EUR 65.8

Ethylenediaminetetraacetic Acid Disodium Salt

E918335 100g
EUR 69
Description: 139-33-3

tri-Potassium Hydrogen Ethylenediaminetetraacetate

15109-82 25G
EUR 22.4

Ethylenediaminetetraacetic acid trisodium salt dihydrate 98%

E08540 100G
EUR 140
Description: CAS N° 150-38-9

Ethylenediaminetetraacetic Acid Tetrasodium Salt Dihydrate

E918310 100g
EUR 57
Description: 10378-23-1

Ethylenediaminetetraacetic acid manganese disodium salt trihydrate

20-abx184692
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  • 500 g

Ethylenediaminetetraacetic Acid

15105-22 25G
EUR 23.1

Ethylenediaminetetraacetic Acid

15105-35 500G
EUR 46.9

Ethylenediaminetetraacetic Acid

15107-02 25G
EUR 25.55

Ethylenediamine-N,N,N’,N’-tetraacetic Acid Disodium Dihydrate

E917735 10g
EUR 253
Description: 6381-92-6

Ethylenediaminetetraacetic-d12 Acid

E917746 10mg
EUR 64
Description: 203806-08-0

Ethylenediaminetetraacetic Acid-d16

E917751 10mg
EUR 75
Description: 203805-96-3

Ethylenediaminetetraacetic Acid Dianhydride

E917595 10g
EUR 58
Description: 23911-25-3

Ethylenediaminetetraacetic Acid Dilithium Salt

E918318 10g
EUR 800
Description: 14531-56-7

Ethylenediaminetetraacetic acid (trisodium salt)

HY-B1009 1g
EUR 43.29
Description: Ethylenediaminetetraacetic acid trisodium salt (EDTA trisodium salt; Trisodium EDTA) is a kind of metal chelating agent (binds to bivalent and trivalent metal cations, including calcium). Ethylenediaminetetraacetic acid trisodium salt has antibacterial, anti-inflammatory, antioxidant, anti-hypercalcemia and anticoagulant activities. Ethylenediaminetetraacetic acid trisodium salt decreases the metal ion-catalyzed oxidative damage to proteins, and allows maintenance of reducing environment during protein purification. Ethylenediaminetetraacetic acid trisodium salt can alleviate the liver fibrosis. Ethylenediaminetetraacetic acid can be used for coronary artery disease and neural system disease research[1][2][3][4][5][6][7].

Ethylenediaminetetraacetic acid trisodium salt

T0423-10mg 10mg Ask for price
Description: Ethylenediaminetetraacetic acid trisodium salt

Ethylenediaminetetraacetic acid trisodium salt

T0423-1g 1g Ask for price
Description: Ethylenediaminetetraacetic acid trisodium salt

Ethylenediaminetetraacetic acid trisodium salt

T0423-1mg 1mg Ask for price
Description: Ethylenediaminetetraacetic acid trisodium salt

Ethylenediaminetetraacetic acid trisodium salt

T0423-50mg 50mg Ask for price
Description: Ethylenediaminetetraacetic acid trisodium salt

Ethylenediaminetetraacetic acid trisodium salt

T0423-5mg 5mg Ask for price
Description: Ethylenediaminetetraacetic acid trisodium salt

Ethylenediaminetetraacetic acid trisodium salt solution

MBS576716-1g 1g
EUR 145

Ethylenediaminetetraacetic acid trisodium salt solution

MBS576716-500mg 500mg
EUR 145

Ethylenediaminetetraacetic acid trisodium salt solution

MBS576716-5x1g 5x1g
EUR 505

(S)-1-(p-Bromoacetamidobenzyl)ethylenediaminetetraacetic Acid (~80%)

B679000 25mg
EUR 193
Description: 81677-64-7

rac (Aminomethyl)ethylenediaminetetraacetic Acid tetra-(t-Butyl Ester)

A616160 250mg
EUR 201
Description: 1391052-55-3

4-Aminobenzyl Ethylenediaminetetraacetic Acid, Tetra(t-butyl) Ester

MBS6078540-10mg 10(mg
EUR 505

4-Aminobenzyl Ethylenediaminetetraacetic Acid, Tetra(t-butyl) Ester

MBS6078540-5x10mg 5x10mg
EUR 2125

rac (Aminomethyl)ethylenediaminetetraacetic Acid tetra-(t-Butyl Ester)

MBS6034066-25mg 25(mg
EUR 610

rac (Aminomethyl)ethylenediaminetetraacetic Acid tetra-(t-Butyl Ester)

MBS6034066-5x25mg 5x25mg
EUR 2600

(S)-1-(p-Bromoacetamidobenzyl)ethylenediaminetetraacetic Acid (BABE)

MBS6041271-5mg 5(mg
EUR 545

(S)-1-(p-Bromoacetamidobenzyl)ethylenediaminetetraacetic Acid (BABE)

MBS6041271-5x5mg 5x5(mg
EUR 2310

(S)-1-(4-Aminoxyacetamidobenzyl)ethylenediaminetetraacetic Acid (AABE)

MBS6025607-5mg 5(mg
EUR 545

(S)-1-(4-Aminoxyacetamidobenzyl)ethylenediaminetetraacetic Acid (AABE)

MBS6025607-5x5mg 5x5(mg
EUR 2310

EDTA Iron(III) sodium salt (Ethylenediaminetetraacetic acid iron(III), Edathamil, Komplexon II)

MBS635476-50g 50g
EUR 345

EDTA Iron(III) sodium salt (Ethylenediaminetetraacetic acid iron(III), Edathamil, Komplexon II)

MBS635476-5x50g 5x50g
EUR 1410

4-(N-Boc-aminoxyacetamido)benzyl Ethylenediaminetetraacetic Acid, Tetra(t-butyl) Ester

MBS6118728-5mg 5(mg
EUR 525

4-(N-Boc-aminoxyacetamido)benzyl Ethylenediaminetetraacetic Acid, Tetra(t-butyl) Ester

MBS6118728-5x5mg 5x5(mg
EUR 2210

Ethylenediaminetetra(methylenephosphonic acid)

E918323 5g
EUR 76

Ethylenediamine-N,N,N’,N’-tetraacetic Acid Calcium Disodium Hydrate

E917590 100g
EUR 161
Description: 23411-34-9

Ethylenediamine dihydroiodide

E08440 500G
EUR 2053.6
Description: CAS N° 5700-49-2

Ethylenediamine Dihydrochloride

15129-22 25G
EUR 16.8

Ethylenediamine dihydrochloride

40000027-1 100 g
EUR 29.69

Ethylenediamine dihydrochloride

40000027-2 500 g
EUR 98.98

Ethylenediamine dihydrochloride

GK6724 100g
EUR 29.04

Ethylenediamine dihydrochloride

GK6724-100 100
EUR 23.8

Ethylenediamine dihydrochloride

GK6724-100G 100 g
EUR 64.8

Ethylenediamine dihydrochloride

GK6724-250 250
EUR 55.4

Ethylenediamine dihydrochloride

GK6724-250G 250 g
EUR 103.2

Ethylenediamine dihydrochloride

GK6724-500 500
EUR 87.1

Ethylenediamine dihydrochloride

GK6724-500G 500 g
EUR 141.6

Ethylenediamine Dihydrochloride

E918353 10g
EUR 69
Description: 333-18-6

N-(1-Naphthyl)ethylenediamine dihydrochl

RM1073-25G 1 unit
EUR 69.21
Description: N-(1-Naphthyl)ethylenediamine dihydrochl

N-(1-Naphthyl)ethylenediamine dihydrochl

RM1073-5G 1 unit
EUR 15.42
Description: N-(1-Naphthyl)ethylenediamine dihydrochl

N-(1-Naphthyl)ethylenediamine dihydrochloride

GK9342 5g
EUR 195.68

N-(1-Naphthyl)ethylenediamine dihydrochloride

GK9342-100 100
EUR 213.7

N-(1-Naphthyl)ethylenediamine dihydrochloride

GK9342-100G 100 g
EUR 294

N-(1-Naphthyl)ethylenediamine dihydrochloride

GK9342-25 25
EUR 79.1

N-(1-Naphthyl)ethylenediamine dihydrochloride

GK9342-25G 25 g
EUR 132

N-(1-Naphthyl)ethylenediamine dihydrochloride

GK9342-5 5
EUR 23.8

N-(1-Naphthyl)ethylenediamine dihydrochloride

GK9342-5G 5 g
EUR 64.8

N-(1-Naphthyl)-ethylenediamine dihydrochloride

NB0650 10g
EUR 84.01

Ethylenediamine dihydrochloride_x000D__x000D__x000D_

E08430 5G
EUR 436.97
Description: CAS N° 333-18-6

CTP disodium dihydrate

561576 100.0mg
EUR 250

CTP disodium dihydrate

MBS5758743-100mg 100mg
EUR 320

CTP disodium dihydrate

MBS5758743-10mg 10mg
EUR 160

CTP disodium dihydrate

MBS5758743-25mg 25mg
EUR 200

CTP disodium dihydrate

MBS5758743-50mg 50mg
EUR 250

CTP disodium dihydrate

MBS5758743-5mg 5mg
EUR 145

CTP disodium dihydrate

MBS3611149-5mg 5mg
EUR 200

CTP disodium dihydrate

MBS3611149-5x5mg 5x5mg
EUR 580

CTP disodium dihydrate

T7881-10mg 10mg Ask for price
Description: CTP disodium dihydrate

CTP disodium dihydrate

T7881-1g 1g Ask for price
Description: CTP disodium dihydrate

CTP disodium dihydrate

T7881-1mg 1mg Ask for price
Description: CTP disodium dihydrate

CTP disodium dihydrate

T7881-50mg 50mg Ask for price
Description: CTP disodium dihydrate

CTP disodium dihydrate

T7881-5mg 5mg Ask for price
Description: CTP disodium dihydrate

N-(1-Naphthyl)Ethylenediamine Dihydrochloride (NEDA) extrapure AR, ACS, 98%

61166 5 Gms
EUR 5.78
Description: Part B

CTP Disodium Dihydrate (>85%)

C831028 100mg
EUR 221
Description: 81012-87-5

Balsalazide Disodium Dihydrate

B116295 10mg
EUR 431
Description: 150399-21-6

E.D.T.A., disodium salt, dihydrate

MB011-1KG 1 unit
EUR 26.7
Description: E.D.T.A., disodium salt, dihydrate

E.D.T.A., disodium salt, dihydrate

MB011-500G 1 unit
EUR 15.27
Description: E.D.T.A., disodium salt, dihydrate

E.D.T.A., disodium salt dihydrate

PCT0105-100G 1 unit
EUR 6.93
Description: E.D.T.A., disodium salt dihydrate

E.D.T.A., disodium salt dihydrate

PCT0105-1KG 1 unit
EUR 31.97
Description: E.D.T.A., disodium salt dihydrate

E.D.T.A., disodium salt dihydrate

PCT0105-500G 1 unit
EUR 17.75
Description: E.D.T.A., disodium salt dihydrate

E.D.T.A., disodium salt dihydrate

PCT0105-5KG 1 unit
EUR 127.76
Description: E.D.T.A., disodium salt dihydrate

ADP disodium salt, dihydrate

MB191-1G 1 unit
EUR 14.92
Description: ADP disodium salt, dihydrate

ADP disodium salt, dihydrate

MB191-5G 1 unit
EUR 72.95
Description: ADP disodium salt, dihydrate

Nitroprusside (disodium dihydrate)

HY-A0119 500mg
EUR 59.52
Description: Nitroprusside disodium dehydrate (Sodium nitroprusside dihydrate) is a vasodilator that available for the research of acute hypertension, heart failure. Nitroprusside disodium dehydrate induces autophagy in glutathione-depleted osteoblasts. Nitroprusside disodium dehydrate acts as a nitric oxide (NO) donor in a rat intestinal ischemia reperfusion model[1][2][3][4].

Nitroprusside disodium dihydrate

T6991-10mg 10mg Ask for price
Description: Nitroprusside disodium dihydrate

Nitroprusside disodium dihydrate

T6991-1g 1g Ask for price
Description: Nitroprusside disodium dihydrate

Nitroprusside disodium dihydrate

T6991-1mg 1mg Ask for price
Description: Nitroprusside disodium dihydrate

Nitroprusside disodium dihydrate

T6991-50mg 50mg Ask for price
Description: Nitroprusside disodium dihydrate

Nitroprusside disodium dihydrate

T6991-5mg 5mg Ask for price
Description: Nitroprusside disodium dihydrate

EDTA Disodium Salt Dihydrate

40500012-1 500 g
EUR 69.53

EDTA Disodium Salt Dihydrate

40500012-2 1 kg
EUR 114.8

EDTA Disodium Salt Dihydrate

40500012-3 2.5 kg
EUR 241.35

EDTA Disodium Salt Dihydrate

40500012-4 5 kg
EUR 411.26

EDTA Disodium Salt Dihydrate

40500012-5 10 kg
EUR 800.11

EDTA, Disodium Dihydrate Salt

20-147 500g/Unit
EUR 73.04
Description: Ultra Pure Grade

EDTA, Disodium Salt, Dihydrate

A2503-1KG 1KG
EUR 46.2
Description: Biotechnology

EDTA, Disodium Salt, Dihydrate

A2503-500G 500G
EUR 26.4
Description: Biotechnology

EDTA, Disodium Salt, Dihydrate

A2503-5KG 5KG
EUR 189.2
Description: Biotechnology

EDTA, Disodium Salt, Dihydrate

CH032 500 g
EUR 195.6

EDTA, Disodium Salt, Dihydrate

CH033 1.0 kg
EUR 130
Description: A reversible metalloprotease inhibitor useful for chelating metal ions.

EDTA, Disodium Salt, Dihydrate

CH034 2.5 kg
EUR 400.8

EDTA disodium salt dihydrate

GE3023 100g
EUR 108.65

EDTA disodium salt dihydrate

GE3023-1 1
EUR 39.4

EDTA disodium salt dihydrate

GE3023-100 100
EUR 11.3

EDTA disodium salt dihydrate

GE3023-100G 100 g
EUR 48

EDTA disodium salt dihydrate

GE3023-1KG 1 kg
EUR 84

EDTA disodium salt dihydrate

GE3023-250 250
EUR 15.9

EDTA disodium salt dihydrate

GE3023-250G 250 g
EUR 55.2

EDTA disodium salt dihydrate

GE3023-5 5
EUR 118.7

EDTA disodium salt dihydrate

GE3023-500 500
EUR 23.8

EDTA disodium salt dihydrate

GE3023-500G 500 g
EUR 64.8

EDTA disodium salt dihydrate

GE3023-5KG 5 kg
EUR 180

EDTA, disodium salt, dihydrate

EB0185 500g
EUR 84.01

EDTA disodium salt dihydrate

GRM1195-100G 1 unit
EUR 6.93
Description: EDTA disodium salt dihydrate

EDTA disodium salt dihydrate

GRM1195-1KG 1 unit
EUR 25.12
Description: EDTA disodium salt dihydrate

EDTA disodium salt dihydrate

GRM1195-500G 1 unit
EUR 13.97
Description: EDTA disodium salt dihydrate

EDTA Disodium Salt Dihydrate

MBS635343-100g 100g
EUR 155

EDTA Disodium Salt Dihydrate

MBS635343-1kg 1kg
EUR 320

EDTA Disodium Salt Dihydrate

MBS635343-25kg 2.5kg
EUR 690

EDTA Disodium Salt Dihydrate

MBS635343-500g 500g
EUR 260

EDTA Disodium Salt Dihydrate

MBS635343-5kg 5kg
EUR 1020

EDTA, Disodium Salt, Dihydrate

MBS4156372-500g 500g
EUR 260

EDTA, Disodium Salt, Dihydrate

MBS4156372-5x500g 5x500g
EUR 920

EDTA, Disodium Salt, Dihydrate

MBS4156373-1kg 1kg
EUR 285

EDTA, Disodium Salt, Dihydrate

MBS4156373-5x1kg 5x1kg
EUR 1095

EDTA, Disodium Salt, Dihydrate

MBS4156374-25kg 2.5kg
EUR 465

EDTA, Disodium Salt, Dihydrate

MBS4156374-5x25kg 5x2.5kg
EUR 1705

EDTA Disodium Salt Dihydrate 0.057M

40500040-1 500 mL
EUR 25.2

EDTA Disodium Salt Dihydrate 0.057M

40500040-2 1 L
EUR 42.6

EDTA Disodium Salt Dihydrate 0.057M

40500040-3 4 L
EUR 110.75

Disodium Molybdate(Ⅵ) Dihydrate

31621-52 25G
EUR 12.25

Disodium Molybdate(Ⅵ) Dihydrate

31621-65 500G
EUR 88.2

Tartaric acid disodium dihydrate

C6836-1000 1g
EUR 48
Description: Metabolic Enzyme/Protease|Phosphatase

Tartaric acid disodium dihydrate

C6836-500 500mg
EUR 40
Description: Metabolic Enzyme/Protease|Phosphatase