We go down to the city with Lloyd, we still have three hours before I must go to the airport in Simferopol for my fellow travelers for the next week. We descend among decaying Tatar houses to the Khan’s Palace, then climb up to Sevastopol Street’s Karaim Synagogue, the muezzin is singing again, sleepy dogs spy on us. A small kitten joins us in our walk, eagerly trotting behind us along the long road, I do not even know where he left us behind.
Autumn in Bakhchisaray
We go down to the city with Lloyd, we still have three hours before I must go to the airport in Simferopol for my fellow travelers for the next week. We descend among decaying Tatar houses to the Khan’s Palace, then climb up to Sevastopol Street’s Karaim Synagogue, the muezzin is singing again, sleepy dogs spy on us. A small kitten joins us in our walk, eagerly trotting behind us along the long road, I do not even know where he left us behind.
Otoño en Bajchisarái
Bajo a la ciudad con Lloyd, aún tenemos tres horas hasta que yo tenga que ir al aeropuerto de Simferopol a recibir a mis compañeros de viaje durante la semana próxima. Descendemos a través de las decadentes casas tártaras hacia el palacio del Khan, luego subimos a la sinagoga karaíta de la calle Sebastopol, el muecín canta de nuevo mientras unos soñolientos perros nos observan. Un gatito se une a nuestros pasos y nos sigue trotando por la larga calle, no me doy cuenta de cuándo desaparece.
magyarul
Oak
Ancora cadrà la pioggia sui tuoi dolci selciati, una pioggia leggera come un alito o un passo. |
on your sweet cobblestones a light rain like a breath or a step Cesare Pavese: The cats will know (en) |
in italiano • auf Deutsch • magyarul
Dissolving: Games
See our post on the early Soviet board games for children
in italiano • auf Deutsch • magyarul
Game
Karpatt, Un jeu (A game). From the CD Sur le quai (2011)
Maman m’a montré un jeu quand j’étais tout p’tit Tu vas voir c’est très marrant on va changer d’pays Chez nous c’est pas facile, notre cabane est en bois On va prendre un bateau y a pas d’place pour papa C’était très rigolo les gens jouaient à tomber dans l’eau Je sais qu’ils faisaient semblant, je l’sais j’suis pas idiot |
Mom showed me a game when I was little: you’ll see it’s so funny, we’re going to change country life is not easy at home, our cabin is of wood we’ll take a boat, there’s no place for Dad. It was fun, people played falling in the water but I knew they were just pretending, I’m not stupid. |
Maman m’a montré un jeu quand j’avais mal au ventre Tu vas voir c’est très marrant on va jouer à attendre Quand on s’ra arrivé tu mangeras tout les jours On gagnera plein d’argent pour faire venir papa un jour De l’autre côté d’la mer, on a couru sur une plage Y avait les sirènes de police on s’est caché sous les branchages | Mom showed me a game when I had stomach ache you’ll see it’s so funny, we play waiting when we’ll arrive, you’ll eat every day we’ll gain lots of money, we’ll also invite Dad one day. On the other side of the sea we ran on the beach the cops blew the horn, we hid under the trees. |
Maman m’a montré un jeu faut s’trouver un abri Tu vas voir c’est très marrant on va camper la nuit Y avait plein d’gens comme nous qui jouaient à cache-cache On s’est fait une cabane dans un tuyau avec des vaches Et puis toute la journée on attendait près des feux rouges On lavait les voitures toutes les voitures avant qu’elles bougent | Mom showed me a game, we have to find a shelter you’ll see it’s so funny, we’ll camp in the night. There were plenty of people like us playing hide and seek, we made a cabin on a field with the cows. Then we waited throughout the day at the red traffic light, we had to wash the cars, all the cars before they move. |
Maman m’a montré un jeu faut s’trouver d’l’argent Tu vas voir c’est très marrant faut tendre la main aux gens Elle rentrait pas souvent, elle travaillait le soir Elle se faisait très belle pour attendre sur un trottoir Moi j’aimais pas trop ça quand elle montait dans les voitures Avec des gars bizarres qui lui faisaient des égratignures |
Mom showed me a game, we have to find money you’ll see it’s so funny, you only have to hold the hand to people she rarely came home, she worked in the night she primped up to go to wait on the street. I did not really like it when she sat in the cars with strange guys who made her scars. |
Maman m’a montré un jeu faut s’trouver des papiers Tu vas voir c’est très marrant on va jouer à s’cacher Les flics nous on trouvé ils ont cogné sur nos têtes Je savais bien qu’c’était qu’un jeu alors j’ai pas fait la mauviette J’ai pas pleuré quand on nous a attaché dans l’fond d’un avion J’ai compris qu’on avait gagné au grand jeu de l’immigration | Mom showed me a game, we have to find papers you’ll see it’s so funny, until then we’ll play hide and seek. The cops found us, they beat our heads. I knew it’s just a game, so I have to persist so I did not cry when they tied us in a plane, I knew we won the big game of immigration. |
in italiano • auf Deutsch • magyarul
Persian afternoon
The song reaching up to the head of the valley
The streets stretching out
The sound of hurrying steps
A man passing by like a dancer in his pleated large trousers
The door wide open to the courtyard, the blue pool, the little dome, equally blue, the pergola
The sound of the water, the voice that preaches, the women in their veils printed with flowers, the song of the cicadas
Neither a wall, nor a mihrab to orientate the prayer, only the room open to the sky, open to the valley, open to the rocks, open to the birds
Open to the south
en français • in italiano • auf Deutsch • magyarul
Après-midi persane
Le chant qui retentit jusqu’au plus creux de la vallée
Les ruelles qui s’étirent
Le bruit des pas qui se pressent
Un homme qui passe tel un danseur dans son large pantalon plissé
La porte grande ouverte sur la cour, le bassin bleu, la petite coupole bleue également, la pergola
Le bruit de l’eau, la voix qui prêche, les femmes dans leur voile imprimé de fleurs, le chant des cigales
Ni mur ni mihrab pour orienter la prière mais la salle ouverte vers le ciel, vers la vallée, vers la roche, vers les oiseaux
Vers le sud
in English • in italiano • auf Deutsch • magyarul
Neu-Braunfels, Texas and its genetic secret
Inset: One of the first issues of Neu-Braunfelser Zeitung, 1852
But first, the “why” question, just how the old-style gumshoe genealogy comes into the high-tech world of modern medical genetics.
Clinical genetic testing for predisposition to the diseases which strike in adulthood (such as breast cancer) retains the classic flavor of a genetic high-risk family study even in 2013. If there is strong family history of cancer, then one gets tested, and learns about one’s personal risk level. But if someone has little or no history of the disease, perhaps because the family was too small, or the patient was adopted, then it’s a real hurdle to get a test. So you can see why people learn all the details about the health of their relatives, and recite all these details when they order tests, and why they get tested together with their family members. Decades ago, this kind of genetic testing was so exotic that it required a determined researcher, or an influential family leader (or both!) to get the ball rolling, and to be a de facto project coordinator.
Luckily, the things have changed. Genetic predisposition testing has become routine, with hundreds thousands people taking tests every year. It also means that the testing in the families has become uncoordinated. Often, people no longer learn the complete picture, who in the family was afflicted with what condition, who else got tested with what kind of a result. And it’s just perfectly fine most of the time. They get a negative or, sometimes, a positive result, and may be all they needed. But then, occasionally, the result defies an easy interpretation, and that’s when the family lore begins to matter. Because unfortunately, not every kind of genetic variation is easy to interpret (and every one of us has millions of genetic variants in our chromosomes!). Usually it’s almost crystal clear if a mutation breaks the gene, or if it leaves all of its important parts completely unperturbed. Sometimes, though, a mutation looks like a not-quite-right part in a complicated machine, kind of like a screw of a wrong size or a wire of a wrong length ... the whole machine doesn’t look obviously broken, but is it going to work now? Mother Nature builds sturdy little machines, where not every little bolt is important!
the New Braunfels Zurcher mutation. Highlighted in yellow is a little “part” (an amino acid
residue) which is replaced by a part of a fairly different shape. Is it going to work now?
Insets: Chart of percentage DNA sharing between relatives (strictly speaking, it’s “autosomal”
DNA only, and percentages for distant relatives are statistical averages rather than
precise numbers); a drawing of the Zurcher family tree with mutation
carriers in black and known cancers in red.
That’s where family connections become really important, and the more distant family, the better. Everyone shares DNA with their relatives; we are 50% genetically identical to our parents, brothers, or sisters, but we share less than 1% of our genes with our 3rd cousins. So if one repeatedly finds the same unusual mutation in a cancer gene in the distant relatives with the same cancer syndrome, then you realize that it didn’t happen by chance. If, whenever you find cancer, you find the mutation, then you can predict who else is at risk (those who carry the mutation), and which relatives are risk-free (because they don’t have the mutation). Charting the elusive family connections isn’t straightforward at all, but finding the missing links may be really rewarding.
The Neu-Braunfels genetic variant story started from the usual family quizzing and family testing – asking patients about their family history, and imploring them to ask their relatives to take this useless genetic tests (and it was practically useless because at the time, nobody could vouch if the variant had anything to do with cancer). Two families responded, and there were a few takers, all close relatives. But we still needed many more test-takers, and more distant relations, by the time the interest abated. The investigation ended without any conclusions.
Fast-forward to 2013. Another young lady have tested positive for the same variant, and I noticed something peculiar. Our new patient lived in the vicinity of San Antonio, Texas, and so did several members of a family which took part in the investigation 2 years earlier. What if...? Yes, in a not-so-few keystrokes I was able to find a 2006 obituary of her grandmother, and to match it with birth and marriage records of the other family (I should mention that most vital records in Texas are available online). The new patient was their 2nd cousin – and neither branch of the family knew that breast cancer was also ravaging the other branch!
But there was more. It didn’t take much longer to realize that all other carriers of this mutation lived in Texas and Arkansas, and to use vital records, census records, and gravestone catalogs to retrace their roots to their shared great-great and great-great-great-
Their story begins in 1821 when Stephen Austin, “The Father of Texas”, first secured a grant to settle 300 Norteamericano families near San Antonio from the colonial authorities of Nueva España who were just about to loose the Mexican War of Independence. Soon, the Viceroyalty of Nueva España turned into Mexican Empire and then to Mexican Republic, but the Anglo settlers kept trickling in, and the conflicts with the Mexican authorities worsened. In 1836 Texas declared independence, and it marked the beginning of a decade or armed hostilities with Mexico and their Comanche Indian allies. The republican government of Texas was desperate to bring in more non-Hispanic settlers, and not just Anglos; they thought that the Germans would do.
On one of the first ships to arrive, the Heinrich, was a 40 years old Alsatian immigrant called Nicholaus Zürcher. The Zürchers were bound for a Medina river settlement of D’Hannis, chartered by a French benefactor of the Republic of Texas; all in all just 27 Alsatian families moved there. Only the old cemetery with a ruined church stands there now, with a number of Zuercher/Zürcher gravestones. They were from a tiny village of Oberentzen in the Haut-Rhin department of Alsace, today’s population 528, just a few kilometers from Rhine, so in the U.S. documents sometimes they called their place of origin France, sometimes Germany; sometimes Mulhouse, sometimes Colmar.
Most of the Zurcher immigrants remained in Medina county with the Alsatians, but Nicholaus’s first wife Magdalena died in 1846, and he struck out for Adelsverein’s German holdings. Prince Carl’s Adelsverein migrants mostly arrived very late in 1844, and there was no way they could reach the Fisher-Miller Land Grant in time for spring planting; worse, the land Prince Carl has bought turned out to overlap with the hunting range of the hostile Comanches! (The whole German Texas story is shaped by unbelievable business ineptitude of the German Noble class as well as by the amazing business acumen of the German commoners; I can’t help thinking that those German migrants must have brought the seeds of the 1848 Revolution with them already!). So the wagon trains from the Carlshafen were too late for their supposed destination; but Prince Carl managed to buy some more land for them mid-way to there – and it happened to be the land which included Comal Springs, the largest freshwater spring in the US South-West and the source of a short but mighty Comal River. This is where Neu-Braunfels (named after Prince Carl’s titular holdings) has spring up – and in just a few years it boasted the best cotton and wool mills in Texas, powered by the raging waters of Comal! Having failed, repeatedly, to organize the immigrant wagon trains on time, Prince Carl was ousted from the Adelsverein, and his successor negotiated a peace and land sharing treaty with the Comanches (the only treaty between Indians and Whites which was never broken by the latter), and finally started moving settlers further inland by 1846. But Neu-Braunfels on Comal Springs was there to stay.
In October 1846 in Neu-Braunfels, Nic Zürcher married Elizabeth Loos, a girl from Hesse-Darmstadt 20 years his junior. They had at least 8 children, the first one, Fritz, born in February 1848 and last, Minna, in 1870 several months after her father’s death. But in this narrative we are following only their 2nd son Emil, born in 1852, because it’s only in his descendants where we know that the BRCA1 mutation can still be found today. In June 1873, Emil Zercher (as he spelled his surname by then) married 18 years old Eliese Gerhardt, who was brought from Prussia to Comal by her parents when she was just one or two years old. Eliese was 75 when she died, so most likely 3 of their children inherited the mutation from Emil rather than from her: Ida, born in 1875, Lina, born in 1878, and Walter, born in 1898.
Ida Zercher Schneider died at 66; it was in her great-grandchildren and great-great-grandchildren, who remained in the New Braunfels area, where we found the initial family connection between the branches of her two grandchildren, Eldred and Erlyne. Lina Acker was one of the last Neu-Braunfelsers to be memorialized in German by a gravestone reading “MUTTER Gest. 1958”, and her daughter, Valeska Schulmeier, gave rise to the Arkansas branch of the family before dying at 62; her son passed the mutation to Valeska’s granddaughters who were struck by the disease. Walter passed on the mutation to his son Clarence Zercher, and among Clarence’s children and grandchildren, breast cancers struck fearsomely early, attacking the young women in their 30s.
“The mystery solved!”, you’d think, “and now everybody got a clear answer”. No, it couldn’t be further from the reality. True, we proved the connection between the mutation and cancer in this family beyond doubt – but the cause-and-effect link is harder to prove. Did the use of a wrong amino acid directly impact the work of the BRCA1 protein? Or was it a mere genetic marker, in itself inconsequential but heralding a different, hitherto unknown mutation somewhere else in this gene? A geneticist can easily see how this mutation may disrupt the protein; indeed it is located in bundle of spiral-like structures knows as alpha helices, and the mutation brings in an amino acid of a different shape which is singularly unsuitable for an alpha-helix. We also know this specific alpha-helix plays a big role in cancer risk; in fact a different mutation just a notch up its spiral even has a popular book written about,
But, you’d think, for the Zurcher descendants, it shouldn’t matter if the mutation is the direct cause of cancer, or a perfectly correlated marker of cancer. Either way, it accurately predicts who is at risk and who isn’t, and isn’t it all what really matters to the patients? And if cancer can’t be avoided, then either way it puts them on track to get new chemotherapy drugs which are only supposed to work when BRCA1 gene is broken? Yes, but ... the experts can’t agree on how to break the news to their physicians. The patient privacy rules are strict, and it’s illegal to disclose personal information of any patient to any other doctor. To say that “we also found this mutation in Jane Doe who is the 3rd cousin once removed of your patient” is to break the law. And if we chose to speak in the most general terms, like, “there is a large family and your patient is one of them, but we can’t disclose any specifics”, then these gynecologists and oncologists might become incredulous, like, “Do you really know it about my patient? Nothing on her chart indicates such an extensive family history! What is your proof?”.
So the genealogy mystery is solved, but the story has no happy end. It is mired in a legal and ethical quandary, and for the scattered descendants of Emil Zurcher, there is no resolution in sight.
magyarul
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