Adriana Jacobs, SM/Prog

ARIAANTJE JACOBS is my 7th great grandmother and I carry the VARIEGATE PORPHYRIA R59W SOUTH AFRICAN MUTATION DIRECTLY FROM HER - MY haplogroup mtDNA U2c1

DATE of death ? Still alive 30 May1728 on inventory ' zijn nagelaten vrouw'

Inventory 30 May 1728

Baptism 1673 Adriana

Her name here should be ADRIANA as the oldest document available

https://www.e-family.co.za/ffy/g5/p5308.htm

a1 Gerrit Janse van Deventer van Veldcamp. Burger Stellenbosch. getroud: 29 Oct 1688 Ariaantje Jacobs, weesmeisie, van Rotterdam. The South African variegate porphyria PPOX R59W could be traced back to Gerrit Jansz van Deventer, born in Veldkamp in the Netherlands, and to his wife Ariaantje Jacobs, who was born in Rotterdam . Her father died when she was 5 months and her mother, when she was eight. Admitted to the orphanage 'Gereformeerd Burgersweeshuis' in Rotterdam, in 1687, the director minister Sewentien decided to send eight of his female orphans (including Ariaantje and her half-sister Willemijntje) to the Cape to become wives of the Dutch settlers. She married Gerrit Jansz van Deventer, and he and his wife had eight children, of whom four had porphyria. The link with this founder family was identified first by Geoffrey Dean, a British physician who settled in South Africa in 1947. He could determine whether Ariaantje or her husband, Gerrit Jansz, carried the mutation. Based on the fact that Hendrik, the son of the half-sister, Willemijntje, also had porphyria the mutation, the carrier was assumed to be Ariaantje.

• ARIAANTJE JACOBS VAN DEVENTER my 7th Great Grandmother

Marriage 1688 -- Adriaantje van Rotterdam

Inventory 30 May 1728 Ariaantje Jacobsz van Rotterdam

WE DO NOT KNOW IF ARIAANTJE (ADRIAANTJIE ARIENS) JACOBS OR HER HUSBAND GERRIT JANSZ VAN DEVENTER CARRIED THIS PORPHYRIA DISORDER/MUTATION, BUT SEVERAL (FOUR) OF THEIR CHILDREN INHERITED IT, AND WITH EACH GENERATION IS HAS BECOME MORE AND MORE COMMON.

The name PORPHYRIA comes from the GREEK ‘porphuros’ meaning reddish-purple.

I have Variegate Porphyria (South African Porphyria) - It’s been in our family for generations (van Niekerks). My grandmother died at the age of 26 – giving birth to her fourth child. She died of kidney failure, due to medication complications…

My DNA test confirmed a positive South African R59W gene mutation – the common defect responsible for Variegate porphyria in South Africa.

That means PORPHYRIC DRUG PRECAUTIONS need to be exercised.

It was done in the PORPHYRIA LABORATORY MRC/UCT LIVER RESEARCH CENTRE (University of Cape Town) by Prof Peter Meissner and Prof Richard Hift.

Prof Richard Hift is now the HOD of the School of Clinical Medicine at UKZN Medical School. They are doing brilliant work in this respect!

• A little bit of Historical background……………..

The South African mutation imported to the Cape in 1685/1688 from Holland and is now widespread in the South African population.

It is also identified in the Netherlands, where it is rare, and shown by haplotype analysis to be genetically related to the South African population. Also the mutation found on one allele of all four South African patients with compound heterozygous ("homozygous") VP.

The South African variegate porphyria gene PPOX mutation R59W could be traced back to Gerrit Jansz (the son of Jan) van Deventer, born in Veldkamp in the Netherlands, and to his wife Ariaentje (daughter of Jacob) van Rotterdam (who was born in Rotterdam).

In the 17th century most people in Netherlands abd Scandinavia did not have surnames but were described as the son of, or the daughter of, the father’s first name.

Gerrit Jansz (the son of Jan) came from Deventer, or rather a suburb of Deventer called Veldkamp.

He was one of the free burghers and came to the Cape in 1685. He was given a grant of land in the Stellenbosch district but he did not have a wife. He must have come from a good family because his grandfather wrote a history of the Dutch-Spanish war.

Ariaentje Jacobs van Rotterdam, (or Ariaantje Adriaansse or Ariaantje van den Berg) father died when she was 5 months and her mother, when she was eight.

• The spelling of Ariaentje varies in different documents. She was admitted to the orphanage ‘Gereformeerd Burgersweeshuis’ in Rotterdam, in 1687.
• The director minister Sewentien decided to send eight of his female orphans (including Ariaentje and her half-sister Willemijntje) to the Cape to become wives of the Dutch settlers. They were sent out on the ship China and arrived in the Cape in 1688.

Four of the female orphans were married within months of their arrival and their names are together in the Cape Marriage Register. One of the four was Ariaantje

Ariaantje (the daughter of Jacob) married Gerrit Jansz van Deventer in 1688, and they and had eight children, of whom four had porphyria. They must have inherited porphyria either from Gerrit or from Ariaantje. It is not known whether porphyria was brought to South Africa by Gerrit Jansz or his wife Ariaantje Jacobs.

Mother of; Jacomina Gerritsz van Deventer, b2 SM; Aletta Gerritsz van Deventer, b4; Johanna Margaretha van Deventer; Jacob Gerrits van Deventer; FOUR OF THE CHILDREN WITH VARIEGATE PORPHYRIA - Dr. Geoffrey Dean - The Porphyrias

Based on the fact that Hendrik, the son of Willemijntje, the halfsister of Ariaantje, also had the porphyria mutation - the carrier was assumed to be Ariaentje.

The details about the parents and grandparents of Gerrit Jansz have been found from the archives in Holland and it would have been possible to trace the ancestry of Ariaantje, from the orphanage in Rotterdam, if only the orphanage records wasn’t destroyed by fire during the bombing of Rotterdam in 1940.

A cluster of porphyria was also identified in a community southeast of Portland, Oregon (Robert Vlietinck, unpublished results).

These people were descended from seven founders who all emigrated to the United States in the middle of the 19th century.

They were endogamous to keep the farming land in the families.

Their ancestry could be traced back to the province of North-Brabant, not far away from the village Veldkamp, where Gerrit Jansz van Deventer was born.

Porphyria Variegate is so ‘common’ in South Africa because one of the early settlers happened by chance to have brought the porphyric gene from Holland and descendants multiplied rapidly.

It appears that the thousands who have inherited porphyria variegate in South Africa are members of this one huge family.

Those who have inherited Variegate Porphyria seem to be more emotional than average and if it wasn’t for modern medicine, porphyria would have done little harm…

Modern medicine, BARBITURATES, SULPHONAMIDES especially and PENTOTHAL are to be absolutely AVOIDED at all costs!!!

In 1939 two medical students at the University of Cape Town; Lennox Eales (Prof Eales) and Jack Chait published the first description on Porphyria in the l’nyanga journal.

The link with this founder family was identified first by Geoffrey Dean, a British physician who settled in South Africa in 1947.

Added by: Cecilia Jacoba Strauss - South African mutation R59W - VARIEGATE PORPHYRIA 4 September 2016

• Weesmeisie van Rotterdam. On 4 August 1688, Willemyntje Ariens de Wit, Anna Elkop, Judith Verbeek, Engeltje Cornelisse van der Bout and Catharina Jansz van der Zee, Ariaante Jansse, Ariaantje Jacobs arrived at the Cape as members of a group of eight orphan girls brought to the Cape following requests by Jan van Riebeeck (1659) and Simon van der Stel (1685) that the Heeren XVII send between 20 and 40 marriageable girls to the Cape. When they were finally sent in 1668 the Heeren XVII wrote that they had been selected because of their experience in farming and it was suggested they be encouraged to marry farmers. They had arrived from Rotterdam on board the China.
• See: http://www.stamouers.com/stamouers/surnames-v-z/532-van-deventer-ge...
• Reference: MyHeritage Family Trees - SmartCopy: Mar 12 2019, 10:53:16 UTC
• Her father died when she was 5 months and her mother, when she was eight. Admitted to the orphanage 'Gereformeerd Burgersweeshuis' in Rotterdam, in 1687, the director minister Sewentien decided to send eight of his female orphans (including Ariaantje and her half-sister Willemijntje) to the Cape to become wives of the Dutch settler.
• https://www.wikitree.com/wiki/Jacobs-4571 (Witness) Arrival: On 4 August 1688, Willemyntje Ariens de Wit, Anna Elkop, Judith Verbeek, Engeltje Cornelisse van der Bout and Catharina Jansz van der Zee arrived at the Cape as members of a group of eight orphan girls brought to the Cape following requests by Jan van Riebeeck (1659) and Simon van der Stel (1685) that the Heeren XVII send between 20 and 40 marriageable girls to the Cape. When they were finally sent in 1668 the Heeren XVII wrote that they had been selected because of their experience in farming and it was suggested they be encouraged to marry farmers. They had arrived from Rotterdam on board the Berg China.
• http://www.e-family.co.za/ffy/g5/p5308.htm Either Ariaantje Jacobs or Gerrit Jansz van Deventer or both of these individuals carried the Variegate Porphyria gene to South Africa. A study by Geoffrey Dean and Hubert Barnes, which included an extensive genealogical investigation, concluded that the strain of Variegate Porphyria found in South Africa is genetically identical in all patients who have the condition, and that they all descend from this couple
• Gerritz Janz van Deventer and Ariaantje were the founding pair of specific porphyria variegata. - confirmed by Prof. Gerhard Geldenhuys. Talk about heredity diseases, Fri 12 Mar 2021 via GSSA electronic webinar.

David Abraham Swanepoel

• https://haplogroup.org/mtdna/rsrs/l123456/l23456/l2346/l346/l34/l3/...

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• Reference: MyHeritage Family Trees - SmartCopy: May 28 2021, 19:57:59 UTC
• Reference: MyHeritage Family Trees - SmartCopy: May 28 2021, 20:04:17 UTC

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GEDCOM Note

Orphan from Rotterdam (Heese Vol 2 p. 67)

• Reference: MyHeritage Family Trees - SmartCopy: Sep 18 2023, 17:37:16 UTC